Amyotrophic Lateral Sclerosis: Pathogenesis

Robert H. Brown Jr; Wim Robberecht

doi:10.1055/s-2001-15260

Seminars in Neurology, Table of Contents

Semin Neurol 2001; 21(2): 131-140
DOI: 10.1055/s-2001-15260

Amyotrophic Lateral Sclerosis: Pathogenesis

Robert H. Brown, Jr¹ , Wim Robberecht²

¹Day Neuromuscular Research Laboratory, Harvard Medical School, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts and
²Department of Neurology, University of Leuven School of Medicine, University Hospital Gasthuisberg, Leuven, Belgium

Abstract

ABSTRACT

Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by motor neuron degeneration. A subgroup of familial cases arises from mutations in the gene encoding cytosolic superoxide dismutase (SOD1). This review considers insight now being gained into ALS pathogenesis from the study of mutant SOD1 protein and its possible mechanisms of adverse effect on nerve cells. Also discussed are the status of other genetic forms of ALS and the elusive question of why this disorder so specifically targets motor neurons. Ultimately, it is hoped that insights from these types of studies will improve the prospects for developing meaningful therapies of ALS.

KEYWORD

Amyotrophic lateral sclerosis

Full Text

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