LPHIA - Doctors have known for years that some people with Lou Gehrig's disease also suffer from a type of dementia. And some with that dementia also develop crippling symptoms like Gehrig's, gradually losing control of their muscles.

Today, a team led by University of Pennsylvania scientists reports the discovery of a likely culprit in both. The two distinct diseases are marked by an abnormal accumulation of the same protein a startling, two-for-one discovery described in the journal Science.

"It's huge," said Mike Hutton, a neuroscientist at the Mayo Clinic College of Medicine in Jacksonville, Fla., who was not involved with the study. advertisement OAS_AD('ArticleFlex_1')


"It completely changes the way in which I think ALS research will focus," he added, using the abbreviation for amyotrophic lateral sclerosis, the disease that felled Gehrig.

With this find, science has now identified at least one faulty protein associated with each of the major neurodegenerative diseases, Alzheimer's, Parkinson's, and Huntingdon's, among others.

Cures remain years away, but pinpointing the apparent culprit is a major step toward fighting it.

The dementia in the new research is a type of frontotemporal dementia, marked by toxic clumps of proteins in the brain's frontal and temporal lobes, which control judgment and behavior.

It is less common than Alzheimer's and tends to strike younger people.

ALS is diagnosed in 5,600 people in the U.S. every year, and up to 30,000 have it at any given time, according to the nonprofit ALS Association. It is almost always fatal.

Scientists aren't certain whether the protein accumulations described in Science directly cause either disease or merely play a supporting role.

The culprit: a previously known protein called TDP-43.

TDP-43 is normally present in the nuclei of healthy brain cells, and is believed to play a role in transcribing the genetic code.
http://www.azcentral.com/news/articl...ehrig1006.html

Six New Members Named to National Neurology Advisory Council

The National Institute of Neurological Disorders and Stroke (NINDS) has appointed six new members to its major advisory panel, the National Advisory Neurological Disorders and Stroke Council. The NINDS, a component of the National Institutes of Health (NIH), is the nation’s primary supporter of basic, translational, and clinical research on the brain and nervous system. NINDS Director Story Landis, Ph.D., formally introduced the new members, who will serve through July 2010, at the Council’s September 14, 2006 meeting.

The National Advisory Neurological Disorders and Stroke Council meets three times each year to review applications from scientists seeking financial support for biomedical research and research training on disorders of the brain and nervous system. Members also advise the Institute on research program planning and priorities. The 18-member Council is composed of physicians, scientists, and representatives of the public. The new members are:

Susan Axelrod, M.B.A., is president and founder of Citizens United for Research in Epilepsy (CURE), a non-profit organization founded by mothers of children with severe epilepsy. Since its inception in 1998, CURE has raised more than $3 million to fund research into finding cures and worked to raise public awareness to the prevalence and devastation of epilepsy. Ms. Axelrod helped organize and cosponsor the NIH conference, “Curing Epilepsy-Focus on the Future,” in March 2000 and is helping to plan the second NIH conference, “Curing Epilepsy 2007: Translating Discoveries into Therapies,” to be held in March 2007.

Lucie Bruijin, Ph.D., is science director and vice president of The Amyotrophic Lateral Sclerosis (ALS) Association. This national non-profit organization is the largest private source of funding for ALS-specific scientific research in the world, having awarded nearly $30 million since 1995. Dr. Bruijin received a bachelor’s degree in pharmacy from Rhodes University, South Africa, and a master’s degree in neuroscience and a doctorate in biochemistry at the University of London. She brings to the Advisory Council a unique combination of scientific skill and experience. She has developed and characterized various model systems of neurodegenerative diseases, including one of the mouse models of ALS.

Ralph G. Dacey, Jr., M.D., is the Henry G. and Edith R. Schwartz Professor and chairman of the Department of Neurological Surgery at Washington University School of Medicine in St. Louis, Missouri. He is also neurosurgeon-in-chief at Barnes-Jewish Hospital. He received his bachelor’s degree at Harvard University and his medical degree at the University of Virginia. Dr. Dacey is secretary of the American Academy of Neurological Surgery, former chairman of the American Board of Neurological Surgery, and a past president of the Congress of Neurological Surgeons. A leader in the field of cerebrovascular neurosurgery, Dr. Dacey’s many honors include the international Grass Foundation award, which recognizes outstanding and continued contributions to research in neurosurgery.

Edgar J. Kenton, III, M.D., is director of the Stroke Prevention Intervention Research Program at the Morehouse School of Medicine in Atlanta, Georgia. This NIH-funded program addresses racial and geographic disparities related to stroke and cerebrovascular disease in the United States and creates community-based prevention and intervention strategies. He received his undergraduate degree from Rutgers University and his medical degree from Cornell Medical College. Dr. Kenton is past president of the American Board of Psychiatry and Neurology and served on the board of directors of the American Academy of Neurology and the American Heart Association. He presently serves as a director on the American Board of Medical Specialties and the Accreditation Council of Continuing Medical Education.

Caroline M. Tanner, M.D., Ph.D., is director of clinical research at The Parkinson’s Institute in Sunnyvale, California. She is also a clinical lecturer in the Department of Health Research and Policy at Stanford University. She earned her medical degree at Loyola University and her doctorate from the University of California at Berkeley. Her many memberships in medical societies include the Movement Disorder Society and American Society for Experimental Therapeutics (both as a founding member). A noted epidemiologist and biostatistician, Dr. Tanner serves on the editorial board of the journal Neuroepidemiology and on the Scientific Advisory Board of the Michael J. Fox Foundation. She fills a vacancy from last year’s Council and serves through July 2009.

Gary Westbrook, M.D., is co-director of the Vollum Institute, a privately endowed research unit of the Oregon Health & Science University. He is also a professor of neurology in the University’s School of Medicine. His research focuses on synaptic transmission in the central nervous system. Dr. Westbrook has clinical training in internal medicine and in neurology. He received his undergraduate degree from Miami University (Ohio) and has degrees in biomedical engineering and medicine from Case Western Reserve University. Among his honors and awards is the prestigious Senator Jacob Javits Award in the Neurosciences. Dr. Westbrook is editor-in-chief of the Journal of Neuroscience.

The National Institute of Neurological Disorders and Stroke is the nation’s primary funder of research on the brain and nervous system. More information about the NINDS and its mission is available at www.ninds.nih.gov.

The National Institutes of Health (NIH) — The Nation's Medical Research Agency — includes 27 Institutes and Centers and is a component of the U.S. Department of Health and Human Services. It is the primary federal agency for conducting and supporting basic, clinical and translational medical research, and it investigates the causes, treatments, and cures for both common and rare diseases. For more information about NIH and its programs, visit www.nih.gov.