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Inclusions of amyotrophic lateral sclerosis–linked superoxide dismutase in ventral horns, liver, and kidney

✍ Scribed by P. Andreas Jonsson; Daniel Bergemalm; Peter M. Andersen; Ole Gredal; Thomas Brännström; Stefan L. Marklund

Publisher
John Wiley and Sons
Year
2008
Tongue
English
Weight
761 KB
Volume
63
Category
Article
ISSN
0364-5134

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✦ Synopsis

Abstract

Mutant superoxide dismutases type 1 (SOD1s) cause amyotrophic lateral sclerosis by an unidentified toxic property. In a patient carrying the G127X truncation mutation, minute amounts of SOD1 were found in ventral horns using a mutant‐specific antibody. Still, both absolute levels and ratios versus wild‐type SOD1 were considerably greater than in other central nervous system areas and peripheral organs. Inclusions of mutant SOD1 were abundant in motoneurons but were also seen in hepatocytes and kidney epithelium. This first examination of mutant SOD1 in both central nervous system and peripheral organs supports the notion that enrichment of misfolded SOD1s might explain the particular vulnerability of motor areas. Ann Neurol 2008

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