Date: Sun, 11 Dec 94 15:09:27 -0500 From: Bob Broedel To: als@huey.met.fsu.edu Subject: ALSD#158 ALS-ON-LINE =============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest (#158, 11 December 1994) == == == == ------ Amyotrophic Lateral Sclerosis (ALS) == == ------ Motor Neurone Disease (MND) == == ------ Lou Gehrig's disease == == ------ Charcot's Disease == == == == This e-mail list has been set up to serve the world-wide == == ALS community. That is, ALS patients, ALS researchers, == == ALS support/discussion groups, ALS clinics, etc. Others == == are welcome (and invited) to join. The ALS Digest is == == published (approximately) weekly. Currently there are == == 420+ subscribers. == == == == To subscribe, to unsubscribe, to contribute notes, == == etc. to ALS Digest, please send e-mail to: == == bro@huey.met.fsu.edu (Bob Broedel) == == Sorry, but this is *not* a LISTSERV setup. == == == == Bob Broedel; P.O. Box 20049; Tallahassee, FL 32316 USA == =============================================================== CONTENTS OF THIS ISSUE: 1 .. Re; Dan Flynn of Boston 2 .. tissue donations from ALS patients 3 .. DECtalk price, etc. 4 .. bulbar 5 .. re: progesterone 6 .. Neurontin 7 .. ANNOUNCE: "The Medical List" 12/94 Update 8 .. positions available 9 .. mouse models (1) ===== Re: Dan Flynn of Boston ========== Date : Fri, 9 Dec 1994 19:32:00 -0500 >From : BillB67399@aol.com Subject: Re: Dan Flynn of Boston Good Morning America plans to show the piece on Dan Flynn of Boston on Tuesday, Dec. 13. the show runs from 7 to 9 a.m. We think the segment will probably run during the early part of the first hour of the show. Regards, Debra Bulkeley (2) ===== tissue donations from ALS patients ========== Date : Fri, 9 Dec 1994 00:43:56 -0500 >From : SPCC977@aol.com Subject: tissue donations from ALS patients My father who is 70 was diagnosed with bulbar onset this May. He is quickly losing all mobility and the end is near. Because of his disease he cannot donate any of his organs to science. However, is there research going on that needs tissue from PALS patients? Where, How and When is this done? He would like to contribute to help find more answers about this disease. Thanks. SPC977@AOL.COM (3) ===== DECtalk price, etc. ========== Date : Thu, 8 Dec 94 10:51:28 CST >From : Woody Bledsoe Subject: Re: DECtalk To Stuart Neilson. I am amazed that your version costs only 200 pounds. I am sure that the DECtalk that I described is a later one (after about August 1994 - Ask Tony Vitale) and a lot more expensive. But very clear in its speech. We also had to pay a consultant to help set up the "command-line" utility (letting a short "name" represent a whole message to be spoken). Thanks for your message. Woody (4) ===== bulbar ========== Date : Thu, 8 Dec 94 11:12:44 CST >From : Woody Bledsoe To : kayOFF@aol.com Subject: Your nice message. Thanks Faye, for your nice message. I am sorry that your mother died of ALS, even in 1974. We never forget them. Yes, I am sure that she would have used a DECtalk machine, and she would have enjoyed it. What is bulbar ALS? I am a scientist, but not a medical one. They tell me that the bulbar strain of ALS, is the one that affects the region around the mouth, throat and lungs. At least that is how my ALS is mainly affecting me. Although, my legs and arms are now beginning to weaken. Not real pain yet, but my "old no good tongue" is so lousy in doing its job, and same for my throat mechanisms, that I cannot talk and can hardly eat without choaking. I have a feeding tube but since I love to eat, I still do most of it through the mouth. Woody (5) ===== re: progesterone ========== Date : 10 Dec 94 16:26:15 EST >From : Wayne Phillips <70303.173@compuserve.com> Subject: re: progesterone Some questions from private email: 1) Did you experience muscle fasciculations (twitching) before progesterone? Yes, they are very pronounced, easily visible. They were my first symptom, in my chest, shoulders, and upper arms, even before muscle weakness. 2) Does the progesterone reduce fasciculations? Not noticably. 3) When were you diagnosed? August 1989 4) Date of first symptoms? January 1989 5) When did you start progesterone? January 1994 6) Any significant loss of strength since then? I haven't taken it consistently; I've been off it a couple of times to see what happens when I stop and to try other things. While I've been on it I've seen definite loss of strength. It hadn't halted my illness, *maybe* slowed it. It may only help symptoms. The best "proof" would be a statisticly significant reduction in mortality. For humans such data would be years in coming, but only months with transgenic mice. 7) Do you know whether the progesterone crosses the blood-brain barrier to enter the CNS or does it just effect muscles? A doc confirmed that steroid hormones (e.g., progesterone) do cross the BBB, both by themselves and when bound by a carrier protien. Nothing I've read suggests that neurosteroids (progesterone, pregnenalone, dehydroepiandrosterone and their metabolites) affect muscles. The recent research that I cited shows that they are synthesized by cells *in the CNS* from cholesterol (same as in gonads and adrenals) in bioactive concentrations (1E-8 M) and modify neurons' responsiveness to other molecules. My unstated assumption was that they do cross the BBB and so oral dosing would/could/ might have an effect. It does. I'm interested in hearing from anyone else who tries it. Wayne Phillips (6) ===== Neurontin ========== THE PRESS-ENTERPRISE Riverside, CA. Volunteers needed for drug study 12/04/94 Loma Linda University is looking for people to take part in a study of a drug that may be helpful in treating Lou Gehrig's disease. The department is taking part in a nationwide trial of the drug, Gabapentin, also known as Neurontin, which has been sold as an anti- seizure drug. For more information, call 799-5037. (7) ===== ANNOUNCE: "The Medical List" 12/94 Update ========== Date : Tue, 6 Dec 1994 21:50:01 LCL Sender : "Clinical WANS: Design & Usage" : >From : Gary Malet Subject: ANNOUNCE: "The Medical List" 12/94 Update *********************************************************************** ANNOUNCEMENT: UPDATE OF AN INTERNET RESOURCE _____________________________________________________ THE MEDICAL LIST A GUIDE TO INTERNET MEDICAL RESOURCES _________________ Version 1.12, 1 December 1994 Dr. Gary Malet, gmalet@surfer.win.net Lee Hancock, LE07144@UKANVM.CC.UKANS.EDU _________________________________________________________ ******************************************************************** A December 1, 1994 update of The Medical List is now available from the Univ. of Michigan Clearinghouse for Subject Oriented Guides URL:gopher://una.hh.lib.umich.edu/11/inetdirs/medclin:malet -------------------------------------------------------------------------- The Medical List is a database of clinical medicine Internet resources. This new and improved version categorizes resources by disease, specialty, and other interest areas and lists more than 45 new entries. It gives text descriptions of resources, spotlights the more developed programs, and gives news and backround on Internet medical resource development. The Medical List is made available in a Mosaic, hypertext, Internet-linked format as "Medical Matrix". Medical Matrix is available at the URL: http://kuhttp.cc.ukans.edu/cwis/units/medcntr/Lee/HOMEPAGE.HTML ************************************************************************** Dr. Gary Malet |7 W 5th Street | "communication, Family Physician |Stockton, Ca. 95206 | search, Healthtel, Inc.-windows based |VOICE 209-466-6878 | delivery, medical telecommunications |FAX 209-466-0502 | networking" gmalet@surfer.win.net |Compuserve 72630.1535| *************************************************************************** (8) ===== positions available ========== >From : Jonathan Marc Rothberg Newsgroups : sci.nanotech Subject : Positions Available, 12 minutes from Yale Date : 6 Dec 1994 17:15:07 -0500 Organization: Curagen Corp. Many positions available. Genomic research, Nanafab projects. Awesome computer science, molecular biology. 12 minutes from yale. Please post, pass on the colleagues. CuraGen Corporation is a dynamic and rapidly expanding biotechnology company on a mission to systematically extract from the human genome those disease related genes for which therapeutics can be successfully designed. CuraGen is pioneering a novel systematic approach to developing pharmaceuticals for treating a broad range of human diseases. In pursuit of its interdisciplinary approach, CuraGen has assembled a research team with expertise in molecular biology, biochemistry, statistical mechanics, computational methods, mathematics, nanofabrication, and spectroscopy. Close ties with several major academic laboratories complement our own resources and facilities. We seek creative and motivated individuals with the dedication and ambition to succeed in an entrepreneurial setting. Send e-mail for a listing of positions. (9) ===== mouse models ========== BIOTECHNOLOGY NEWSWATCH Monday December 5, 1994 MOUSE MODEL MAY LEAD RESEARCHERS TO LOU GEHRIG'S DISEASE MECHANISM By: Marjorie Shaffer Nearly two years after gene hunters bagged one of their biggest prizes -- the long sought gene for inherited cases of Lou Gehrig's disease, researchers are still puzzling over the exact mechanisms that lead to the degeneration of motor neurons in the brainstem and spinal cord, causing muscle wasting, paralysis and death. While it is still not known how the SOD mutation causes ALS, scientists recently got a lucky break in their quest for an answer: mice. Fortunately, mice transfected with the mutant human gene for SOD develop symptoms of ALS, including a shuffling, dragging gait and increasing paralysis. The discovery is leading to new ways of studying the disease. ``The lack of an animal model had been a huge obstacle,'' said Mark Gurney of Northwestern Medical School, who was the first researcher to introduce the mutant gene into mice. ``But we got lucky. The mutant gene that causes the disease in humans, causes the disease in mice. And the disease evolves in only about four months in mice.'' Gurney believes that the aberrant SOD gene involves ``a gain-of-function mutation,'' in which the enzyme adds a function that makes it toxic to motor neurons. Rather than causing some change in the actual biomechanical makeup of SOD itself, the missense mutation appears to affect the exterior structure of the backbone of the protein, he said. That structural change appears to transform the function of the enzyme. But it isn't clear what that new function exactly is, nor whether anti-oxidants would have any affect. Other researchers are gathering evidence that abnormalities in neurofilaments, the structures that form the scaffold of neurons, lead to the death of motor neurons. In affected neurons, these neurofilaments swell along axons near the cell body. Jean-Pierre Julien at McGill University and co-workers at Johns Hopkins have created transgenic mouse models that contain multiple copies of human neurofilament gene, leading to over-expression of the protein. These mice also show signs of ALS. ``The excess of neurofilaments leads to the destruction of the transport system of the axon, and the axon degenerates and then the muscle degenerates,'' Julien said. His group screened the DNA of 536 ALS patients and found five who possessed mutations in the genes for neurofilament. ``It is possible that the accumulation of neurofilaments can provoke neuronal cell death,'' he said. The next step will be to transplant the mutated genes found in these patients into mice. === end of als 158 ===