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Duke University Medical Center Durham, NC (Drs. Siddique, Pericak-Vance, Hung, end Roses. Mr. Phillips, Ms. Warner, Ms. Speer, and Ms. Siddique).
University of Wisconsin, Madison, WI (Dr. Brooks)
University of Chicago, Chicago, IL (Dr. Roos)
Montreal Neurological Institute, Montreal, PQ, Canadas (Dr. Antel)
Tufts-New England Medical Center, Boston, MA (Dr. Munsat)
Johns Hopkins University School of Medicine, Baltimore, MD (Dr. Bias)
Familial amyotrophic lateral sclerosis (FALS) constitutes 5 to 10% of cases of ALS and, in most families, its inheritance is consistent with an autosomal dominant trait with age-dependent penetrance. The biochemical abnormality underlying the disorder is unknown. We analyzed DNA from 131 Members of 6 multigenerational ALS families, which included 13 affected Members, for genetic linkage to 39 expressed and DNA markers, using the techniques of 2-point linkage analysis, multilocus linkage analysis, and exclusion mapping. We identified FALS families with structures suitable for linkage, by computer simulation techniques. A DNA bank established to provide optimum use of available FALS families provided DNA from immortalized lymphoblast cell lines and frozen postmortem tissue. We could not link FALS to any of the markers studied, but excluded chromosome regions unlikely to be a locus of the FALS gene. With the help of this exclusion data, we will concentrate on regions of the human genome that remain unexcluded.
Address correspondence and reprint requests to Dr. Siddique, Division of Neurology, Box 2900, Research Park Building #1, Room 102, Duke University Medical Center, Durham, NC 27710.
Supported by grants from the NIH (K07 NS00960-04 and P01 NS21442-04) (T.S.) and the Amyotrophic Lateral Sclerosis Association (T.S.), as well as from NIH grants NS19999, AG05128-03 (A.D.R.), and R01 S23008-03 (M.P.V.), the Muscular Dystrophy Association of America (T.S. and A.D.R.), and the Denver Fund (A.D.R.).
Received December 2,1988. Accepted for publication in final form February 2.1989.
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 T. Siddique ALS: Molecular Clues to the Jigsaw Puzzle of Neuronal Degeneration Cold Spring Harb Symp Quant Biol, January 1, 1996; 61(0): 699 - 708. [Abstract] [PDF]
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 H. Deng, A Hentati, J. Tainer, Z Iqbal, A Cayabyab, W. Hung, E. Getzoff, P Hu, B Herzfeldt, R. Roos, et al. Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase Science, August 20, 1993; 261(5124): 1047 - 1051. [Abstract] [PDF]
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