New findings are a window into the late stages of 'Lou Gehrig's' disease

February 4, 2004 - BETHESDA, MD -- Amyotrophic lateral sclerosis (ALS) may be better known to some as "Lou Gehrig's disease," a reference to the great New York Yankees hitter and teammate of Babe Ruth who was struck down by the disease. Some 30,000 Americans now suffer from this disabling, almost invariably fatal, disease that is caused by the degeneration of the body's motor neurons, muscle atrophy and weakness.

No one knows what causes the process of terminal neurodegernation to begin. However, using a two-step process to analyze 6,800 genes, scientists have now found an ALS-specific "signature" in spinal cord gray matter taken from ALS patients. The researchers were also able to distinguish familial ALS (FALS) from sporadic ALS (SALS). They believe their approach can reveal some of the distinct changes that underlie the terminal stages in the disease.

A New Study

The new study is entitled "The Molecular Signature of Late-Stage Human ALS Revealed By Expression Profiling of Post-Mortem Spinal Cord Gray Matter." The authors are Fernando Dangond and Sandra Camelo, Laboratory of Transcriptional and Immune Regulation, Center for Neurologic Diseases, Brigham and Women's Hospital, Harvard Medical School, Boston, MA; Daehee Hwang, Gregory Stephanopoulos and George Stephanopoulos, of the Bioinformatics and Metabolic Engineering Laboratory, Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, MA; Piera Pasinelli and Robert H. Brown, Jr., of the Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, MA; Matthew P. Frosch, from the C.S. Kubik, Laboratory of Neuropathology, Department of Neuropathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA; and Steven R. Gullans of the Laboratory of Functional Genomics, Department of Neurology, Harvard Medical School, Boston, MA. Their findings are published in the January 2004 edition of Physiol

Contact: Donna Krupa
American Physiological Society

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