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Year 2000 Hunt-Diser of The Year Awardee

Hunt-Dis has a larger membership then any support group or chapter in the USA with over 350 members. Last year we initiated an annual award to be given at the HDSA annual convention each year  to the person we, collectively, felt had contributed to most to awareness, research,  fund raising for in HD.  In 1999 the first award, was given to Barbara Boyle, Director of the HDSA, for  raising awareness for HD throughout the country. 

The award is universal and non-technical (ie not for doctors, researchers,etc  who are recognized by HDSA) for a person who has contributed the most to  promoting HD awareness  in the past year.   The award reads "Presented in recognition of outstanding contributions in the effort to cure Huntington's Disease" 

I am pleased to announce that MARIE NEMEC  has been voted the Year 2000 Hunt-Diser  of the Year award!!!!

CONGRATULATIONS MARIE!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

Following are the list of  nominees who are all to be given a BIG THANK YOU
from the bottom of all our hearts for their unending efforts in promoting HD awareness!!!!

Marie Nemec - for her commitment to a cause which has never touched her family in arranging and participating in bicycling for a CURE for HD  in 1999 and 2000 cycling across the country.  Marie has helped raise awareness about HD, as well as funds for research for a cure,  in small towns all across America..  She is considered one of  our most   unselfish and dedicated HD Warriors working towards raising HD awareness. 

Jim Pollard for his tireless dedication to HD families through his past and present work as nursing home Administrator in homes that work with HD families, and his continued travels around the US and in other countries educating nursing homes and HD chapters about caring for someone with HD. He is also the co-author of a very well put together guide for caring for late stage HD patients. 

Ardie Newbold and Lou Wilkenson -Both have been an inspiration  and an excellent educator with their incredibly eloquent writings and thoughts  helping not only those with Huntington's but caregivers too, to realize  that there can indeed still be life and even joy  and laughter  after diagnosis. Both Ardie and Lou have taken the time to open their soul about how someone with HD feels in order to help others cope and understand from a person with HD's perspective. with pHDs.

Joan Radamacher for the unconditional love, help, assistance she gives to everyone touched by HD.  It was described that meeting Joyce is like being touched by an angel.

Tom Caldwell.  who consistently provided excellent information and compassionate advise to families living with HD. 

Honorable Mentions:

Rob Laycock and  Renette Davis ..many people from around the world found help at their websites when seeking information on Huntington's, at a time when very little information was available.  These two people, both affected by
HD in their lives, revolutionized  HD on the internet with  their websites. In less than a generation, families went from no information available  to more accurate information than one can manage. 

Carmen Leal for her dedication to promoting HD awareness by traveling and speaking around the country at HD conventions, support groups and to just about anyone who will listen, mostly at her own expense.    Carmen wrote "Faces of Huntington" and helped produce the "Faces" CD, both of which make
contributions to both HD Research and the Kelly E. Miller Juvenile Fund, and will be beginning a new book about children with HD in the very near future. ---Carmen indicated her desire to be removed from the list of candidates.

Jean Miller  for her tireless work over the years on the hunt-dis list, providing loving support  for people caregiving Juvenile HD's, researching scientific documents and providing information to the HD community. Jean has offered  support and friendship as well  as scientific information to all families living with HD. 
 ---Jean indicated her desire to be removed from the list of candidates.


Mouse Model Suggests Huntington's Disease May Be Reversible

WESTPORT, Apr 19 (Reuters Health) - When expression of a transgene encoding the protein responsible for Huntington's disease was turned off in a mouse model, the animals failed to develop the characteristic neuropathology or progressive motor dysfunction of the disease.

The authors of the study conclude in the March 31st issue of Cell that "a continuous influx of the mutant protein" is necessary for maintenance of symptoms, suggesting that Huntington's disease may be reversible. 

Dr. Rene Hen and colleagues of Columbia University in New York, New York, created a conditional transgenic mouse model of Huntington's disease by using the tetracycline-responsive gene system, which enables expression of a transgene to be turned off with tetracycline analogs. The mice carried both a chimeric mouse/human exon 1 of the huntingtin gene with a polyQ expansion and beta-galactosidase (lacZ) reporter sequences.

The mice exhibited the mutated huntingtin fragment as well as other hallmarks of Huntington's disease, including neuronal inclusions, characteristic neuropathology andprogressive motor dysfunction, Dr. Hen and colleagues report.

To turn off expression of the polyQ-huntingtin transgene, the investigators treated mice with 2 mg/mL of doxycycline for 16 weeks. The doxycycline completely inhibited expression of the transgene as evidenced by lacZ staining.

Less than 1% of striatal neurons from treated mice stained positive for nuclear huntingtin at 34 weeks compared with 60% in untreated mice. Intra- and extranuclear aggregates also disappeared in the doxycycline group. 

Dr. Hen's group observed "a clear arrest of neuropathology in the gene-off mice," and a halt to the progressive loss of D1 receptors that was seen in the untreated or gene-on mice. The doxycycline group also exhibited a reversal of the motor dysfunction to levels, in some cases, that were comparable to healthy, control mice.

"Turning off the expression of polyQ-huntingtin...results in either a halt or a reversion of the different phenotypes," Dr. Hen and colleagues say. "This implies that irreversible changes that commit the cell to neuronal dysfunction or death have not necessarily taken place."

The authors add that "therapeutic approaches aimed either to destroy or inactivate the mutant huntingtin protein might be effective." They further note that "the elucidation of the mechanisms responsible may provide new targets for therapeutic interventions for patientssuffering from Huntington's disease and other neurodegenerative disorders."

Taken from the American Academy of Neurology.


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