Journal
ARCHIVES OF NEUROLOGY
Volume 68, Issue 8, Pages 1057-1061Publisher
AMER MEDICAL ASSOC
DOI: 10.1001/archneurol.2011.178
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Funding
- National Institutes of Health [NS050641, AG13854, T32 AG20506]
- Les Turner ALS Foundation
- ALS Association
- Vena E. Schaff ALS Research Fund
- Harold Post Research Professorship
- Herbert and Florence C. Wenske Foundation
- David C. Asselin MD Memorial Fund
- Les Turner ALS Foundation/Herbert
- Florence C. Wenske Professorship
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Background: Mutations in optineurin have recently been linked to amyotrophic lateral sclerosis (ALS). Objective: To determine whether optineurin-positive skeinlike inclusions are a common pathologic feature in ALS, including SOD1-linked ALS. Design: Clinical case series. Setting: Academic referral center. Subjects: We analyzed spinal cord sections from 46 clinically and pathologically diagnosed ALS cases and ALS transgenic mouse models overexpressing ALS-linked SOD1 mutations G93A or L126Z. Results: We observed optineurin-immunoreactive skeinlike inclusions in all the sporadic ALS and familial ALS cases without SOD1 mutation, but not in cases with SOD1 mutations or in transgenic mice overexpressing the ALS-linked SOD1 mutations G93A or L126Z. Conclusion: The data from this study provide evidence that optineurin is involved in the pathogenesis of sporadic ALS and non-SOD1 familial ALS, thus supporting the hypothesis that these forms of ALS share a pathway that is distinct from that of SOD1-linked ALS.
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