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August 31, 2003
PETA Delayed
I know I promised the PETA article today but I've taken a personal day to spend time with friends and enjoy the holiday weekend. I'll have the finished PETA article tomorrow!
Posted by Dave at 09:09 PM | Comments (0) | TrackBack
August 30, 2003
Terrorists Threaten Medical Research
No, not Islamic terrorists…Animal “Rights” Terrorists.
Yesterday a terrorist group called the ‘Revolutionary Cell’ claimed responsibility for exploding two bombs outside Chiron, the third largest biotechnology company in the United States. Researchers at Huntingdon Life Sciences have had their homes damaged and one researcher was beaten up so badly they needed to go the hospital. A few weeks ago one of their warehouses was firebombed.
For years, extremists in this country (and other countries) have been targeting animal research and not just with picketing and lobbying. They’ve been using bombs, arson and physically attacking researchers.
The one of the main argument of these terrorists is that we do not need animals to do medical research. This is argument is patently false. Take a look at the medical studies that are being done for Huntington’s Disease. You’ll see that fly’s, mice, and worms are the most commonly used animals. When you read the studies you’ll see why animals are needed to do the research.
Researchers are trying to identify exactly how the HD gene causes the early death of neural cells and test potential therapies to prevent this early cell death. Seeing the effects of how genes are altered from one generation takes only weeks. With humans it would take decades between each generation. With mice it only takes researchers a few weeks to follow the progression of Huntington’s Disease, whereas with humans it would also take decades. In addition, they can quickly test promising chemicals that may slow or stop the progression of the disease.
So why do the animal rights terrorists risk the lives of humans? Because they believe that an animal life is the equal of a human life. It could even be argued that many in the animal rights movement see animals of deserving more legal rights than humans. To them the injury and death of a few humans is much smaller crime than the death of lab animals.
You can follow a running list of animal rights terrorism on this seemingly pro-terrorism website.
Here is what one prominent animal rights terrorist says in the animal rights magazine “Bite Back”:
“Yet terrorism is simply another means of warfare. Terrorism is the means of warfare utilized by the less dominant forces against the more dominant. In other words, when you can't afford aircraft carriers, nuclear attack submarines, nuclear weapons, and uranium bullets, you deploy suicide bombers, hijackings, assassination and sabotage.”
Another quote: “We did not sink those ships for you or for any of the six billion hominid assholes on this planet. We sank them for the whales.”
And another: “When liberating chickens, it only matters what the chickens think or feel. The feelings and thoughts of the chicken farmer are irrelevant. The feelings and thoughts of the armchair critics are irrelevant. The feelings and thoughts of the media are irrelevant. Only the chickens matters, or the whales, or the minks, or the chimps, or the trees, depending on the action.”
Still another: “I personally cannot get overly worked up about deprivation of human rights in a world where non-humans have no rights at all. Until animals, plants, rivers, and wetlands have rights, none of us have any rights at all because without eco-systems and without diversity, rights are meaningless.”
And another: “The world will be a much nicer place without us. But if we can buy time for other species and for eco-systems and if some of us can alleviate the suffering inflicted on other species, then this is a worthwhile pursuit.”
More: “Whites, Blacks, Indians, Asians, etc are all the same - we are all a bunch of self centered, over self glorified conceited naked apes, all divine legends in our own mind and all confused in our pathetic little primate brains about what this world is all about.”
And: “Don't bring any more humans into being. There are enough of us.”
Tomorrow: PETA.
Posted by Dave at 09:45 AM | Comments (0) | TrackBack
August 29, 2003
Generations Of Hope Approved
I just got word from Traci of the 'Generations of Hope Ranch Camp' that they have received approval on their 501(c)(3). That means that all donations to this worthy cause are fully tax deductable.
If you are not familiar with the Ranch Camp you can read past articles here. This organization is going to provide two important services to the HD community. First, they are establishing a camp where families dealing with Huntington's Disease can get a break and be in an understanding and accepting environment. Second, they are setting up a support line where HD families can call when they just need somebody to talk to.
Both of these things are needed in the HD community. You can contribute by visiting this page on their website.
Posted by Dave at 09:29 AM | Comments (0) | TrackBack
Needed: Normal Brains
I thought that headline would get your attention.
As we've mentioned before, the Harvard Brain Bank does important research on Huntington's Disease. I know a number of pHD's who are planning to donate their brains to the Brain Bank.
According to this article in the National Geographic, Harvard has a shortage of "normal" brains. To understand how Huntington's Disease uniquely affects the brain, researchers need to compare it to a brain that is not affected by HD. According to the article, Harvard gets 240 diseased brains a year but only 30 'normal' brains.
If you aren't at risk for Huntington's Disease, your donation can also make a difference. Talk to a neurologist about how donate. It could make a difference.
Posted by Dave at 09:11 AM | Comments (0) | TrackBack
August 28, 2003
Important Lou Gehrig's Discovery
This discovery may have important implications in Huntington's Disease research.
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's Disease, is a protein aggregate disease much like Huntington's Disease. Researchers from the University of Texas Southwestern have identified a mutant protein called SOD1 that causes the early death of spinal cells. They found aggregates (clumps) within the SOD1 protein.
Further, the researchers found that while other areas of the body is capable of processing the mutated SOD1, the spinal cord can't. The researchers then tested various chemicals found in the body, but not in the spinal cord. They eventually found chemicals that removed the aggregates from the SOD1, reversing the ALS process.
What does this have to do with HD? They found the Mutated SOD1 protein Huntington's Disease (and Alzheimers & Parkinsons). This could have very interesting implications for future HD research.
Posted by Dave at 09:43 AM | Comments (0) | TrackBack
August 27, 2003
NaPro Make A Bold Move
The pharmaceutical industry is riskier than investing in a Madonna movie.
Boulder, Colorado based Napro Biotherapeutics has sold their only commercial product so that they can invest heavily in developing new products. This sale gives them over $70 million to pay of debts and fund research.
One area that NaPro is specializing in is gene editing therapy where they take portions of DNA and attempt to repair cells. They are currently working on applying this gene editing therapy to Huntington's Disease and Sickle Cell Anemia.
Next year NaPro will start clinical trials on their most promising therapies. Let's hope their HD therapy is one of them.
Posted by Dave at 08:20 AM | Comments (0) | TrackBack
August 26, 2003
Medicaid Update
I've been scouring the Internet looking for good sources of information on the Medicare drug coverage bill that Congress is haggling over. So far I haven’t had much luck.
Most seniors lobbying groups, trying to save their members money, are pushing for the most expansive drug program possible. States are pushing for expanded Medicare coverage (which is paid by the federal government) so that they can reduce their Medicaid costs (which are paid by state governments). Budget hawks, looking at the federal deficit, are advocating no new spending and politicians up for reelection next year are eyeing the large block of voters who qualify for Medicare. Insurance and pharmaceutical companies have their own takes on this also.
All of these groups provide information about the Medicare bill, but none provide comprehensive information on all benefits and consequences of the bill. The news media hasn’t been any better. It’s hard to condense 1000 pages of detail into a 20-second news spot or column. Since few are willing to read each of the bill variations, the coverage has often relied on information provided to them by the various interest groups. Not much help there.
So why should anybody who is not on Medicare care? Simple. This bill will affect insurance costs, drug costs, and drug development for everybody.
Where do we stand now? Separate bills have passed the House and the Senate. There are some significant differences between the two bills. The House and Senate is haggling out these differences in a Conference Committee. Each side is trying to create a compromise that will be acceptable to all. If they succeed in reaching a compromise, that compromise version will then back to the House and Senate for a vote. If both sides pass the same identical bill then it will go to the President for his signature (and he will sign it).
So far, the Conference Committee has been unable to come to a compromise. Some pundits are now putting the odds of the bill successfully clearing the Conference Committee at only 50/50. I suspect a bill will make it out of the Conference Committee and it will be signed by the President before the end of the year. Congress is heading into a break, so expect to see of ads in the upcoming weeks urging voters to lobby their congressmen and senators.
From what I see now, any Medicare bill that is likely to make it out of the Conference Committee is likely to do the HD community more harm than good. Stayed tuned here for information on any new developments in this story.
Posted by Dave at 08:08 AM | Comments (0) | TrackBack
August 25, 2003
HealthCentral Gets It Wrong
HealthCentral.com has an article that is a quick translation of a study abstract. In explaining Huntington's Disease they write:
"The age of onset and severity depend on how many copies of the faulty gene are replicated on chromosome 4."
Oops. Those with HD only have one faulty gene. The age of onset and severity varies depending on the number of CAG repeats. I sent them a note. We'll see if they fix it.
UPDATE: I got an email back. Their doctor who handles the comments is on vacation until September 3rd.
UPDATE: I've forward my concern to a person in their business office.
Posted by Dave at 07:49 AM | Comments (0) | TrackBack
August 24, 2003
Can We Get A Translator?
“Here we report that Htt interacts with ND via HAP1, and that MLK2 phosphorylates and stimulates the activity of ND. Furthermore, we show that Htt and HAP1 facilitate the activation of ND by MLK2. To our knowledge, ND is the first example of a neuron-specific transcription factor involved in neuronal development and survival whose activity is modulated by Htt. We propose that Htt, together with HAP1, may function as a scaffold for the activation of ND by MLK2.”
Huh??? When I started this blog…
One of my goals was to provide readable explanations of what researchers, scientists, and doctors (the “white coats”) were saying. It ain’t easy. The English language, while vast, is inaccurate. To get around this limitation, white coats have developed their own language in order to talk to each other. The above excerpt is a good example.
When I saw this study abstract I knew I had a problem. It would take a small book to explain each of the terms used in the abstract, describe their relationship, and explain why this study is relatively important. So what am I going to do?
Skip to the conclusion…
The University of Colorado study has provided some important clues on how the huntingtin protein (the protein affected by HD) is used in the building of brain cells. This type of research not only helps identify potential ways to treat Huntington’s Disease, it also provides information about neural cells that could benefit a wide range of neurological diseases and conditions (such as paralysis).
This study is another example of how money spent on HD research also advances research in so many other areas.
Here’s the study abstract:
Proc Natl Acad Sci U S A. 2003 Aug 5;100(16):9578-83.
Stimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2.
Marcora E, Gowan K, Lee JE.
Department of Molecular, Cellular, and Developmental Biology, University of Colorado, Campus Box 347, Boulder, CO 80309, USA.
NeuroD (ND) is a basic helix-loop-helix transcription factor important for neuronal development and survival. By using a yeast two-hybrid screen, we identified two proteins that interact with ND, huntingtin-associated protein 1 (HAP1) and mixed-lineage kinase 2 (MLK2), both of which are known to interact with huntingtin (Htt). Htt is a ubiquitous protein important for neuronal transcription, development, and survival, and loss of its function has been implicated in the pathogenesis of Huntington's disease, a neurodegenerative disorder. However, the mechanism by which Htt exerts its neuron-specific function at the molecular level is unknown. Here we report that Htt interacts with ND via HAP1, and that MLK2 phosphorylates and stimulates the activity of ND. Furthermore, we show that Htt and HAP1 facilitate the activation of ND by MLK2. To our knowledge, ND is the first example of a neuron-specific transcription factor involved in neuronal development and survival whose activity is modulated by Htt. We propose that Htt, together with HAP1, may function as a scaffold for the activation of ND by MLK2.
PMID: 12881483
Posted by Dave at 09:52 AM | Comments (2) | TrackBack
August 23, 2003
Study Backs Minocyline
In a study worth examining, researchers took a closer look at minocycline and its effects on two different HD mouse models.
They found that minocyline protected the cells in these mouse models two different ways. This is the best study to date to look at just how minocycline affects cells affected by Huntington's Disease.
Based on this study, pHD's would be highly encouraged to continue taking minocycline. Interestingly, a study mentioned here just recently concerning R6/2 mice showed that while minocycline was beneficial to protecting cells in the brain it did not have an affect on the behaviors of the mice. (Original article)
The results of these two studies will need to be compared and more mouse studies need to be conducted. There has been documentation of some HD patients dying from HD without significant cell death and this has not fully understood yet.
So where we are left is...minocycline may or may not help you. However, its a relatively safe medication and no HD study has shown that it causes harm. It's definitely worth staying on minocycline until more questions have been answered. As always, consult your doctor before making any changes. They know you best.
Posted by Dave at 08:09 PM | Comments (8) | TrackBack
August 22, 2003
Have You Got A Hole In Your Head?
This has gotten some coverage but this article by ABC News is more reader friendly than most I've seen. Researchers are trying an experimental procedure in Parkinson's patients where they insert viruses (actually a safe virus segment) with a genetic code that will hopefully fix a genetic disease - gene therapy.
Because of the blood-brain barrier, they are inserting the virus through a small hole in the skull. Thus the 'hole in your head' mention. What they find out from this experiment will be closely followed by HD researchers who are also trying to deliver gene therapy to the brain.
You can read the article here.
Posted by Dave at 09:46 AM | Comments (0) | TrackBack
Genetic Research At UW
The Seattle Times today has an interesting interview with one of the leaders of the Human Genome project - Robert Waterston. From the article:
"Waterston, who turns 60 next month, came to the University of Washington in January to lead the department of genome sciences, which has three of the eight recognized leaders of the genome project. Last week, he broke ground on a $150 million state-of-the-art building to house his department and that of bioengineering."
You can read the article here.
Posted by Dave at 09:35 AM | Comments (0) | TrackBack
August 21, 2003
No Blogging Today
Even though I started the day at 4am, it just ended with getting back late from an HD meeting. I'll post a fresh update tomorrow. Go check out Jerry's HD Lighthouse, he's got several new items on the front page.
Posted by Dave at 10:49 PM | Comments (0) | TrackBack
August 20, 2003
New HD Mouse
Researchers have announced the creation of a new transgenic HD mouse. This one has a CAG count of around a 140. The R6/2 mouse, the one most commonly used in HD research has a CAG count in the 70's. Using the best mouse model is important in analyzing study results. The R6/2 mouse model had fit nicely into 12 week studies.
As mentioned in this abstract, researchers from UCLA have created a new HD mouse. In this mouse they have found that dopamine seems to have a major factor in HD symptoms. This really isn't too surprising considering the positive effect anti-depressants can have on HD-positive patients. The question is...is the new mouse representative in what happens in humans or is what they've observed only happen in this version of HD mice?
I'm all for the creation of new HD mice. The better the mouse model, the sooner we'll have an effective treatment or cure.
Posted by Dave at 07:23 PM | Comments (0) | TrackBack
August 19, 2003
Mixed Feelings About Amarin
A Rick Stewart interview is now available on The Wall Street Transcript. Stewart is the CEO of Amarin Pharmaceuticals, the only company to have a Phase III trial drug in the works specifically for the treatment of Huntington's disease. Later this year, he is going to be given the prestigious Guthrie Award by HDSA for his support of Huntington's Disease research.
That all sounds great so why do I have mixed feelings? Well, because I believe at times he's acted more like a CEO than a researcher. A CEO's job is to ensure the survival of the company. The CEO must manage cash flow, attract investment and select what products the company will develop and support. A researcher is interested in useful discovery and accurate information. These two jobs have very different objectives as the CEO's job is to paint a pretty picture for investors. This involves highlighting the good and sweeping under the bad in making the sales pitch for the company.
The SEC and the FDA carefully watch what pharmaceutical companies say to ensure that a certain amount of honesty is retained in corporate communications.
Unlike what many people believe, not all pharmaceutical companies are rolling in money. Development costs are huge, development times are long, and there is a limited timeframe where the developer has exclusive rights to sell the drug. Startup companies eat through a lot of cash trying to develop marketable and profitable drugs. A startup CEO’s primary job is to ensure that there will be enough money available to ensure the survival of the company.
Last year, Amarin was running out of money. This is not an unusual situation for young pharmaceutical companies. To drum up interest, Amarin issued a press release last October that stated that LAX-101, their Huntington’s drug, had shown positive results in the Phase III trial and that they were in discussions with the FDA. This was followed by a barnstorming tour by the CEO with the financial press to attract investors. This was important to Amarin as their stock had been dropping since late 2001, when it was nearing $30 a share, to around $2.15 a share just 8 months later. If the stock dropped much lower the company would be at risk of being delisted by NASDAQ which could kill the financial future of the company. It worked. Over the next few weeks the stock price had doubled to nearly $6 a share.
On January 28th of this year, Amarin announced that they had raised $21.2 million in a stock sale to a group of investors. Timing is everything in business and it was definitely on Amarin’s side this time. The next day, January 29th, Amarin found out that the FDA would not approve LAX-101 and would require another Phase III trial before giving approval. With questionable wording, the press release that followed five days later called this "further progress". We should all be so lucky to have progress like that. The stock is now trading at $2.62 and the share price is still dropping.
Amarin has done nothing illegal. If you carefully read their press statement from last October they do state the results did not reach a statistical significance for the entire patient population. This is a big ‘red flag’ for knowledgeable investors as the FDA isn’t likely to approve under this circumstance and in this case they certainly didn’t.
However, the HD community is not comprised of knowledgeable investors. The HD community is comprised of individuals who are looking for some hope for themselves and their families. A lot of hope was raised and then dashed a few months later. This is why I have mixed feelings about Amarin. They had to have known that the odds were against LAX-101 being approved, but they didn’t say this to the HD community.
Fortunately for us, we can get access to the active ingredient in LAX-101. It’s purified EPA which can be found in fish oil and its sold over the counter. Taking enough fish oil to get 2 grams of EPA a day will be approximately equal to what is in LAX-101. So even if LAX-101 isn’t approved or if Amarin goes broke, we can still get the active ingredient thanks to the neutraceutical industry.
Amarin has done what it’s needed to do to keep the company alive. They are working to bring an HD drug to market. They are also making the case to investors that it is possible to make money by developing drugs for Huntington’s Disease. I am grateful for these things. I am not grateful for the false hopes that were raised and dashed in the process.
As I said...”Mixed Feelings”.
Posted by Dave at 09:50 AM | Comments (3) | TrackBack
August 18, 2003
World Congress On HD Postponed
This bites...
I checked in this morning to see what the news was out of the World Congress on Huntington's Disease. It was scheduled for last weekend in Toronto. After nearly being cancelled or moved earlier in the year due to the SARS panic, the convention was finally killed at the last minute due to the regional power outages.
The World Congress is comprised of HD organizations from around the world. Organizations involved include HDSA, Hereditary Disease Foundation and the Huntington Study Group. Scheduled speakers included such notables as Dr. Nancy Wexler, Dr. Alice Wexler, and Dr. Gillian Bates. The topics were to wide ranging and presenters were to be available to discuss 92 different study abstracts.
There's no word on when the event will be rescheduled.
Posted by Dave at 02:02 PM | Comments (0) | TrackBack
August 17, 2003
A Sad Day In The Blogosphere
Warning: This post has nothing to do with Huntington's Disease.
It's a sad day.
A friend of this blog is going through a difficult time. Venomous Kate’s Electric Venom blog has been a support of the HD Blog since the beginning. She has provided support, advice, and even some code for this blog. As far as I know, she did this without even knowing what Huntington’s Disease is. Because of her kindness and generosity, she is the only non-HD, non-medical website that I link to. She is one of the reasons this blog is what it is today. She’s a class act.
So what happened? First a little background. A common element in the blogosphere is the use of wit and repartee. Kate plays off this trait with her ‘Venomous’ moniker. At times the blogosphere’s wit and repartee is lighthearted and silly such when Frank J. blames Glenn of blending puppies. At other times the wit can get razor-sharp and nasty. The blogosphere has become a virtual Algonquin Round Table.
Like in the real world, with blogs misunderstandings can happen and feelings do get hurt. Sometimes this leads to a war between blogs where all stops are pulled out and people get hurt. A few weeks back, one blogger was reported fired from her job when a reader from another blog called her employer.
So what does this have to do with our friend Kate? A daughter she loves dearly did something she shouldn’t have done…she read her mom’s email. Her 12-year-old daughter found some private correspondence that, to her, indicated her mom had been attacked (the aforementioned wit & repartee). As 12-year-olds are want to do, she acted without thinking. Believing she was defending her mom, the daughter posted a nasty and very un-ladylike comment on the other blogger’s website – dagoddess.com. It wasn’t hard to trace the comment back to Kate’s house.
This is where the ugly part of the story begins. Other people posted rebuttal comments protecting Joanie (dagoddess). Some charged Kate with faking everything, being a drunk, and with being an abusive parent. So that I’m being clear here…Joanie didn’t say these things, people commenting on her blog did.
If it had stopped here things might have blown over quickly, but somebody then did something really stupid. Some anonymous reader (who will now be referred to as ‘idiot’) of the websites escalated the situation and decided to call child protective services on Kate and accuse her of drunken child abuse. This idiot, probably in some drunken stupor, thought he/she was protecting Joanie and now some people no doubtedly believe that Joanie made the call.
It’s extremely unlikely that it was Joanie, these types of actions are typically done by individuals who feel they have no other option. Joanie has the ‘power of the press’. If she chose to, she has the option to use her blog to attack and respond. It would even raise her readership. Frankly, it does her no good to make an anonymous (and illegal) attack. Thanks to ‘guilt by association’ now this (probably drunken) idiot has unintentionally smeared Joanie for something she almost certainly wasn’t involved in and wouldn’t condone.
Now a lot of people have been hurt and this is what makes it a sad day. Kate, who was been cleared by Child Protective Services, feels attacked and has been hurt by all this. Joanie has been attacked and hurt by Kate’s daughter and readers who feel she is responsible. Supporters of both bloggers have been attacked, especially those tarnished by the actions of the one idiot. Now there is a lot of anger, hate, and mistrust. All of this because of a child’s stupid mistake and an idiot’s vicious action.
This is a sad day for all.
UPDATE: Fixed horrible misspelling of Joannie's name.
UPDATE: Joni of JoniElectric.com would like it to be known she IS a four-letter word (and not Joannie).
Posted by Dave at 08:45 AM | Comments (9) | TrackBack
August 16, 2003
Brainy About the Brain
The fine folks at Stanford's HOPES have developed a neat little brain tutorial specifically for those interested in Huntington's Disease.
This animated tutorial is easy to follow with drawings and animation. If you'd like a better understanding of just what goes on in our brains this is a great place to start.
Click Here To Display The Tutorial.
Posted by Dave at 01:43 PM | Comments (0) | TrackBack
August 15, 2003
Medicare Madness
Keep an eye out on what happens with Medicare over the next few weeks. Congress has a chance to fix one of their screwups from last year. In that session they voted to cut physician reimbursement by almost 10% over two years.
Because of this doctors are dropping out of the plan reducing options for patients. Check out this article, Congress may correct this error.
Posted by Dave at 11:01 PM | Comments (0) | TrackBack
August 14, 2003
Generations Of Hope Camp
The fine folks at the Generations Of Hope Ranch Camp have added pictures of the proposed camp.
They are also working on an exciting fundraiser for later in the year. Keep tuned for details.
You can see the camp photos here.
Posted by Dave at 07:42 PM | Comments (0) | TrackBack
August 13, 2003
Creatine Boosts Brain
In a non-HD study just published in 'Proceedings: Biological Sciences', creatine increased memory recall and brain function by as much as 20%.
Creatine. It's not just for athletes anymore.
Posted by Dave at 06:49 PM | Comments (0) | TrackBack
August 12, 2003
Lithium May Improve HD Symptoms
The Brain Research Bulletin has a study where researchers tested lithium on HD mice. Lithium did not prolong the lives of the mice but it did improve their "motor peformance". This might be a useful medication for those with movement problems.
Here's the abstract:
Brain Res Bull. 2003 Aug 30;61(4):375-83.
Chronic lithium chloride treatment has variable effects on motor behaviour and survival of mice transgenic for the Huntington's disease mutation.
Wood NI, Morton AJ.
Department of Pharmacology, University of Cambridge, Tennis Court Road, CB2 1PD, Cambridge, UK
Expression of the Huntington's disease (HD) mutation in mice (R6/2 line) causes a progressive neurological phenotype that includes deterioration of motor function resembling that seen in HD. The current study sought to determine whether or not chronic treatment of R6/2 mice with lithium chloride would have an effect on the progression of the phenotype, in light of lithium's reported neuroprotective and anti-depressive properties. Treatment began either before or after the onset of symptoms. Chronic treatment with lithium caused a significant improvement in rotarod performance when treatment was started post- but not pre-symptomatically. There was no overall effect on survival in either group, but further analysis revealed that in the post-symptomatic group, mice could be assigned to one of two distinct groups, depending on the effects of lithium. One subgroup of mice lost weight faster, died earlier and showed rotarod performance similar to the vehicle-treated controls. The other subgroup lost weight at a normal rate, died at a similar age, but showed greatly improved motor performance compared to controls. The improvement in rotarod performance suggests that lithium may improve motor symptoms as well as depression in some HD patients.
PMID: 12909280
Posted by Dave at 10:44 AM | Comments (0) | TrackBack
August 11, 2003
Treating Ourselves
Here's a nice little article from the Glascow, Scotland Herald.
The article is about a conference later this month on the use of over-the-counter supplements such as fish oils to treat brain disorders. They mention that the US market for supplements is now over $36 billion a year. In the conference they will be discussion how fatty acids are being trialled for Huntington's Disease.
You can read the article here.
Posted by Dave at 08:37 PM | Comments (0) | TrackBack
August 10, 2003
At Risk
If you haven’t been to the Huntington’s Disease Advocacy Center (HDAC) website you’re missing one of the better HD websites. Its chock full of articles and information.
One of the latest articles is a piece written by Jill Ginsburg. She’s a woman who has been ‘at risk’ and found out she had breast cancer. She expresses the surprise of so many who’ve lived with the specter of Huntington’s Disease their whole life only to find, like anybody else, that they can also get other diseases.
In her case the news is mostly good. The cancer is being treated and she tested ‘negative’ for HD. Now at the age of 44 she’s living her life and she’s now training to enter the Danskin Triathlon.
It’s a good read, check it out.
Posted by Dave at 09:26 AM | Comments (0) | TrackBack
August 09, 2003
HDDW Update
HD Lighthouse has posted the August update for the Huntington's Disease Drug Works (HDDW).
Dr. Goodman's update contains a wealth of information on HDDW and it's role within the HD community.
This is a Must Read Article. You can check it out here.
Posted by Dave at 09:01 AM | Comments (0) | TrackBack
HD Benefiting From The HD Positive
The South Delta Leader (Delta, British Columbia) has very nice article on Dean Crain. If that name sounds familiar, it’s because his family is featured this month’s Oprah Magazine.
Here’s what Dean has to say about living with Huntington’s:
"It's not a death sentence unless you make it that way."
Dean has difficulties walking but he raises money for HD research by participating in distance running events. It’s not easy… he has to wear gloves to protect his hands when he falls, a frequent occurrence.
Dean’s fundraising has been very successful; so far he has raised $30,000 for the British Columbia chapter of the Huntington Society of America.
His positive attitude is evident throughout the article which finishes with this:
“After being diagnosed with the disease, Crain made a list of things to look forward to instead of dwelling on the things he would lose.
"It's is getting shorter every day," he says of the list.”
You can read the article here.
Posted by Dave at 08:44 AM | Comments (0) | TrackBack
August 08, 2003
More On HD Murder-Suicide
An interesting article on the aftermath of the Culp murder-suicide. The gist of the article is to calm any worries people might have regarding Huntington's Disease. A couple of money quotes:
There's no reason to be fearful," he said.
"I've followed a number of people with Huntington's and there was absolutely no reason to be fearful in any of those cases about potential harmful behavior to others."
All-in-all, a well balanced article written by a responsible journalist. The doctor does a good job but gives a very low estimate of the occurence of HD (1 in 20,000).
Warning: There is a graphic desciption of Linda Culp's cause of death contained in the article.
You can read the story here. More on this case here, here, and here.
Posted by Dave at 09:27 AM | Comments (0) | TrackBack
Brock University Receives HD Grant
Brock University has announced that they have received a $102,578 grant from the Canada Foundation for Innovation to expand their involuntary movement program to include Huntington's Disease, multiple sclerosis, and cerebral palsy.
Posted by Dave at 09:12 AM | Comments (0) | TrackBack
Decade Of The Brain
Something a little different today...
This is an editorial written by Dr. Silvia Helena Cardoso of Brain and Mind. It's a nice background piece that talks about the different types of scientists involved in brain research.
Here's a portion of the editorial:
"Molecular neurobiologists explore neurons' gene stock material searching for clues about the structures in the brain's molecules. Breakthroughs in molecular genetics show great promise of yielding methods to treat and prevent diseases such as Huntington's disease and muscular dystrophies. "
Posted by Dave at 09:06 AM | Comments (0) | TrackBack
August 07, 2003
Minocycline Not Helpful in HD Mouse Study
Dr. Gillian Bates, Dr. Emma Hockly and the rest of the group at King's College in London have come out with yet another HD study.
Minocycline has been one of the promising treatments for slowing the progression of Huntington's Disease. Its safety has been proven with decades of use. Minocycline is available in generic form so it's inexpensive, it crosses the blood/brain barrier and it’s known function on cells makes it a logical choice for HD patients.
However, according to this study in the Annuls of Neurology researchers found that minocycline and doxycycline did not alter the behavior of the HD mice and they did not alter the protein aggregates (clumps) in the neural cells.
With no clear benefit and the alway's possible risks due to medication side effects the researcher's recommend caution in going forward with human studies of minocycline.
If you are taking minocycline for HD, do not stop taking it without having talked to your doctor first . This is a relatively safe medication and your doctor may feel it's in your best interest to stay on it.
Here's the study abstract:
Ann Neurol. 2003 Aug;54(2):186-196.
Minocycline and doxycycline are not beneficial in a model of Huntington's disease.
Smith DL, Woodman B, Mahal A, Sathasivam K, Ghazi-Noori S, Lowden PA, Bates GP, Hockly E.
Department of Medical and Molecular Genetics, King's College London, United Kingdom.
Huntington's Disease (HD) is an inherited neurological disorder causing movement impairment, personality changes, dementia, and premature death, for which there is currently no effective therapy. The modified tetracycline antibiotic, minocycline, has been reported to ameliorate the disease phenotype in the R6/2 mouse model of HD. Because the tetracyclines have also been reported to inhibit aggregation in other amyloid disorders, we have investigated their ability to inhibit huntingtin aggregation and further explored their efficacy in preclinical mouse trials. We show that tetracyclines are potent inhibitors of huntingtin aggregation in a hippocampal slice culture model of HD at an effective concentration of 30microM. However, despite achieving tissue levels approaching this concentration by oral treatment of R6/2 mice with minocycline, we observed no clear difference in their behavioral abnormalities, or in aggregate load postmortem. In the light of these new data, we would advise that caution be exercised in proceeding into human clinical trials of minocycline. Ann Neurol 2003
PMID: 12891671
Posted by Dave at 07:17 AM | Comments (0) | TrackBack
More On Creatine
Check out this great piece that Jerry wrote on HD Lighthouse. If you're still have some questions or confusion about creatine this article may clear it up for you. Incuded in the article is a clear guide on possible side effects and information on the creatinine test. You can read the article here.
Posted by Dave at 06:45 AM | Comments (0) | TrackBack
August 06, 2003
Couple Reunited In Death
From the Elkheart (Michigan) Truth:
Last Friday Dennis Swindle passed away from Huntington's Disease. The night before a tear rolled out of his eye when he had learned that his ex-wife was unable to visit him. His brother, Blair Swindle said "We told him Debbie wasn't going to make it and he just seemed to give up."
One day later, Debbie Swindle died from cancer. Debbie's fiance, Terry Rohyans, and the two families decided on a joint funeral the two will be buried next to each other in matching caskets.
You can read the whole story here.
Posted by Dave at 08:20 AM | Comments (0) | TrackBack
Huntington's Disease Triathlon
Last Sunday the 12th annual Huntington's Disease Sprint and Olympic Distance Triathlon was held in Key Biscayne, Florida. This event is sponsored by the South Florida Chapter of HDSA. You can read about this fundraiser here.
Posted by Dave at 08:05 AM | Comments (0) | TrackBack
August 05, 2003
Lawsuit Filed in HD Murder Case
The estate of Linda Culp has filed an $18 million wrongful death lawsuit against the estate of Gary Culp. As covered here and http://www.huntingtons.info/MT/archives/000041.html earlier, Linda Culp was murdered a couple of weeks ago and her husband, Gary Culp, was the prime suspect. He fled the state and was later found dead in what was believed to be a suicide. Gary Culp had been diagnosed with Huntington's Disease several years earlier and had been showing more symptoms of the disease.
So why are they suing? On the surface it does seem a bit silly since the decendants are due to receive the estate and now legal costs will eat into the value of the estate. This is actually a smart financial move. Only a portion of the estate is protected from estate taxes, but financial awards from a lawsuit are entirely tax-free (since they are consided a reimbursement for loss). In addition, an insurance company is probably on the hook should the Gary Culp estate lose. If they win, this will result in a lot more money for the family.
You can read more on this here.
Posted by Dave at 08:37 AM | Comments (0) | TrackBack
August 04, 2003
Antidepressant's May Protect Brain
Another study, conducted by Washington University Medical School, demonstrates that antidepressants may protect the brain. In this study referenced on scienceblog.com, found woman who took antidepressants had less shrinkage in the hyppocampus than depressed women who did not take antidepressants. As was mentioned here a few days ago, HD also affects the hippocampus.
The researchers are now looking into whether the antidepressants just protect the brain from damage or if they also help restore the brain.
Posted by Dave at 09:31 AM | Comments (0) | TrackBack
HDSA Guthrie Awards
HDSA has announced their annual Guthrie Awards Dinner which will be held this year at the Waldorf-Astoria Hotel in New York on October 2, 2003.
Honorees include John Mellencamp, Columbia University's Dr. Stanley Fahn, Amarin's CEO Rick Stewart, and the San Diego Charger's CEO Dean Spanos & wife Susie Spanos. (I hope the Spanos bring along Bill & Romana Johnston.)
For the rest of the information here's the press release:
NEW YORK, Aug. 4 - The legacy of folksinger Woody Guthrie and his wife Marjorie will be commemorated at the Huntington's Disease Society of America's (HDSA) seventh annual Guthrie Awards Dinner. The gala will be held at the Waldorf Astoria Hotel on Thursday, October 2, 2003, beginning with a cocktail reception at 6:00 p.m.
This year's honorees are John Mellencamp, world-renowned singer, songwriter and musician, who will receive the Woody Guthrie Award for his embodiment of Woody Guthrie's ideals; Stanley Fahn, M.D., noted Professor of Neurology and Chief of the Movement Disorders Division at Columbia University, who will receive the Guthrie Family Humanitarian Award for his compassion and concern for the care and support of people with Huntington's Disease; Rick Stewart, Chief Executive Officer of Amarin Corporation plc, who will receive the Marjorie Guthrie Leadership Award for outstanding humanitarianism in the corporate world; and Dean Spanos, President and CEO of the San Diego Chargers and his philanthropist wife Susie Spanos, who will receive the Harold Leventhal Community Service Award for their commitment to raising awareness about Huntington's Disease. Judy Collins, celebrated folk artist, will reprise her role as Mistress of Ceremonies.
When Woody Guthrie lost his battle with Huntington's Disease (HD) in 1967, his wife, Marjorie, founded what is now known as the Huntington's Disease Society of America with a mission to promote and support research to find a cure for HD, help those affected by the disease and their families and educate the public and healthcare professionals about HD.
Huntington's Disease is an inherited, degenerative brain disorder that results in the progressive loss of control of both the mind and the body. Each child of an affected parent has a 50-50 chance of inheriting this deadly disease. Presently, there is no effective treatment or cure for HD, but with the help of HDSA, there is hope. The Huntington's Disease Society of America is committed to making this the last generation with HD.
All proceeds from the Guthrie Awards Dinner benefit the Woody and Marjorie Guthrie Research Fund to find a cure for Huntington's Disease. For more information or to purchase tickets/tables, please contact the Huntington's Disease Society of America at 800-345-HDSA.
Posted by Dave at 09:22 AM | Comments (0) | TrackBack
August 03, 2003
Beating HD - Scott Midyett
If you don't regularly visit the HD Lighthouse, you should. They have just posted a wonderful article written by Scott Midyett. It’s a must read.
Scott is a pHD who started having HD symptoms eight years ago. His symptoms had progressed to the point that he could no longer work. At one point, he visited the emergency room six times over a two month period due to broken bones from falls.
So far the story isn't that unusual, except...
The picture at the beginning of the story is of Scott riding a unicycle...recently. As you read his story you'll find that he's completed a tri-marathon and that his CT scans actually show his brain getting healthier!
Now the story is unusual! So why is his health improving when for others it is declining? The answers are all in the story.
First, he got good help. Scott had a good doctor. The doctor put Scott on an anti-depressant right away. Depression is one of the deadliest (and most common) symptoms of HD. It causes people to give up hope and in many cases even commit suicide. When Scott started taking the Paxil he was taking his first important step in taking care of himself.
Second, he kept himself informed. Scott kept up with HD research by reading the HD Lighthouse. There he found out about the brain was able to regenerate neural cells and ways he could help that process.
Third, Scott didn't give up. When life got difficult, he started to fight back. After his doctor gave his approval, Scott started walking.
Fourth, he believed in the 'impossible'. He believed that if he worked at it, he could improve. Scott believed he still had some control over his future.
Fifth, he didn't give up. This is the hardest step. The second biggest reason that people fail is because they quit. They just stop. There had to have been times when he was sick or tired or the situation looked a little more hopeless but he kept at it. Over time he got stronger and he got healthier.
Oh, what's the biggest reason people fail (I know you're wondering)?
They don't start.
Sixth, Scott understood the importance of "small steps" and this is the secret to making big changes in your life. He focused on achieving the "next step". His first goal was walking on the treadmill. Then his goal was going faster and from there each goal was a "small step" further. Do you think he would have succeeded if his initial goal, after recovering from his many falls, was to ride a unicycle? His road from there would have been filled with the potholes of frustration and despair; instead his road was paved with accomplishments. Small steps.
Seven, Scott doesn't stay satisfied. He keeps looking for the next challenge. With each challenge that he conquers he gains pride, self-esteem, and motivation to do it again.
So can you do this also? Of course you can! Read Scott's story and follow the seven steps he took to success. It could become your first step to getting healthier and happier.
Posted by Dave at 10:24 AM | Comments (1) | TrackBack
August 02, 2003
Coenzyme Q10
Coenzyme Q10 (coQ10) is an over-the-counter supplement that many neurologists are recommending to their Huntington’s Disease patients.
Coenzyme Q10 may sound like a funny name for a supplement but for scientists the name is very descriptive. A ‘coenzyme’ is a substance that helps enzymes do their job (enzymes are types of proteins in the body that speeds up chemical reactions). The Q10 portion of the name identifies part of the chemical structure. There are many other coenzymes (Q1-Q9 for example) but coQ10 is the only one that is regularly sold as a supplement.
So what does it do? Well, coQ10 plays several roles in our body. Our cells use it to produce energy and the body also uses it as an antioxidant. An antioxidant protects cells from chemicals called ‘free radicals’ that cause damage to important portions of cells, including the DNA.
It is these properties of coQ10 that make it interesting to HD researchers and doctors. The HD gene causes the creation of a protein clump (amyloid) with the neural cells. This ‘clump’, causes the neural cells to become increasingly inefficient and eventually die too soon. By having HD patients supplement with coQ10, doctors are trying to help the neural cells stay healthy longer. This would have the effect of slowing the progression of the disease.
Ok, so this sounds good in theory. Is there any actual evidence that it does help those with HD? Yes, there is good evidence that it mildly slows the progression of the disease. A randomized, double-blind study done by the Huntington’s Study Group (HSG) in 2001 showed a 13% slowing of the progression of the disease for those who took coQ10 (300mgs/day). A mouse study published in 2002 showed an increase in survival of 17%. These aren’t huge numbers but, as one researcher involved in the HSG study stated, it could buy another 18 months (or more) for someone with HD.
One thing that is becoming increasingly clear in the HD research community is the importance of determining the proper dosage for a potential treatment. In some cases, such as creatine, a higher dosage has been shown to be significantly helpful while a lower dosage had no effect at all. There is now interest in finding out if a higher dosage of coQ10 would be more effective in HD patients. This was the case in a Parkinson’s study where those who received the highest dose (1200mgs) showed the most benefit – a 44% slowing in the progression of the disease.
One comment on animal studies: There have been a number of problems in conducting animal studies with coenzymes such as coQ10. In many cases the results have been affected by the animals diet, by how the animal utilized (or didn’t utilize) other enzymes, unexpected consequences to altering various genes or finding that some animals synthesis and utilize coenzymes differently than expected. This (coQ10) is one area where human studies are very important in determining potential benefits, risks and proper dosages. Hopefully we’ll see more in the near future.
Before you run out and buy coQ10 you should first consult your doctor (which you should do anytime you start something new). There are some known interactions with other drugs (I’m not aware of any that are considered ‘serious’). Some people also experience some unpleasant side effects (some which are alleviated by taking coQ10 with food). You may also wish to visit the HD Lighthouse and read what it has to say about coQ10. They have a different perspective on this and have several negative comments regarding the supplement and do not appear to support its use. (It’s never good to rely on just one source for information, including the HD Blog website.)
If you’ve ever purchased coQ10 you quickly find out that it is very expensive. This is because it is very expensive to manufacture. The cost for coQ10 may significantly drop in the not-to-distant future. A new manufacturing process has just been developed that is much less expensive. As the process comes into use, the price should start to drop significantly.
At this point, I believe coQ10 is worth considering if your doctor approves. While there is some evidence (mixed at this point) that healthy people may not benefit from coQ10 there have been several human studies that have shown a benefit to those suffering from various diseases (especially those involving the mitochondria - the center of cells) including Huntington’s, Parkinson’s, cancer, heart disease and diabetes. The benefit from taking coQ10 (if any) isn’t likely to be ‘huge’ as it appears to mildly slow the progression of HD, but it could help buy you the time you need to eventually benefit from a future ‘cure’.
Posted by Dave at 10:09 AM | Comments (1) | TrackBack
August 01, 2003
Another Piece of the Puzzle
Emery University Researchers are getting a clearer picture of how the HD gene is causing neural cells to die early. In 1995, Dr. Xiaojiang Li identified a protein in certain neural cells which would eventually be called HAP1 (huntingtin-associated-protein-1). Dr. Li found that the huntingin protein interacts with this protein and though it isn’t fully understood it’s believed it might be involved in growth-signaling within the cells.
Last year, Dr. Li’s laboratory developed a strain of mice that lacked the HAP-1 protein and started examining what was then happening in the hypothalamus of the mice. What they saw was the neural cells in the hypothalamus dying in the same manner (apoptosis) as the neural cells die in HD patients. Since the HAP-1 protein interacts with the Huntingtin protein, the doctors then decided to check HD mice to see if the same thing was happening in the hypothalamus. This turned out to be a smart thing to do. The hypothalamus in the HD mice was experiencing the same type of neural cell death as they saw in the HAP-1 deficient mice.
So the doctor’s did the next logical thing, they added HAP-1 to cell cultures from the HD mice to see what would happen. They found that the additional HAP-1 kept the neural cells from dying early.
This research is another important piece of the HD puzzle. The gene was only identified in the early 1990’s. The resulting protein clumps were only found and identified in the mid-1990’s. Since then, researchers have been trying to identify the function of the huntingtin protein in the neural cell and identify what proteins/enzymes interact with the huntingtin protein. In addition, they are trying to identify just how the mutated huntingtin protein changes this interaction.
In the last 18-21 months as researchers have learned more details about what goes on within a neural cell, they have been coming to a consensus that the protein clumps are the cause of the cell death (as opposed to a reaction to something else going on). (Counterpoint: Dr. Li in this piece states that this is still unclear.)
This recent research is so important because understanding exactly how the hungtingtin protein clumps cause the early cell death gives researchers clear paths to research ways to counter the early cell death. Coupling this research with the relatively new cell-arrays has allowed researchers to cut years if not decades in finding drugs that will effectively treat Huntington’s Disease. As a result certain classes of chemicals are looking particularly promising and have shown encouraging results in animal testing.
This study does not mean that we can start taking HAP-1 pills tomorrow. What it means is that researchers can now test various methods for increasing HAP-1 levels in neural cells. As usual, this means finding drugs that will cross the blood/brain barrier and affect the targeted areas or finding some other method of increasing HAP-1 levels. This will take some time. This new found information will also help speed up other HD research as each piece of the puzzle brings the full picture clearer into focus.
You can find the Emory University press release on this finding here.
Posted by Dave at 08:52 AM | Comments (0) | TrackBack