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Title of Journal: J Neurol

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Abbravation: Journal of Neurology

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D. Steinkopff-Verlag

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DOI

10.1016/0022-0736(91)90007-9

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1432-1459

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Specific electron transport chain abnormalities in

Authors: Jerry Lin Andrew Diamanduros Soheli A Chowdhury Stephen Scelsa Norman Latov Saud A Sadiq
Publish Date: 2009/02/25
Volume: 256, Issue: 5, Pages: 774-782
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Abstract

In an amyotrophic lateral sclerosis ALS patient who also had an IgA gammopathy autopsy studies identified the IgA in the surviving motor neurons Further the IgA bound the surface of isolated bovine motor neurons and inhibited neuronal proliferation in culture To determine the pathologic basis of this IgA interaction with motor neurons a neuroblastoma cDNA library was generated and screened with the IgA monoclonal antibody Reactive clones were identified as flavin adenine dinucleotide FAD synthetase To extend this finding to ALS in general quantitative RTPCRs were performed on blood samples from 26 ALS and 30 control blood samples to determine mRNA expression levels of FAD synthetase and other electron transport chain proteins specifically riboflavin kinase RFK cytochrome C1 CYC1 and succinate dehydrogenase complex subunit B SDHB All expression levels were measured against a control enzyme glyceraldehyde3phosphate dehydrogenase GAPDH Expression levels for a nonrespiratory chain protein betaactin were also measured We found that FAD synthetase expression levels were decreased in ALS samples compared to expression levels in controls P = 00151 Expression levels for RFK CYC1 and SDHB were also significantly decreased in the ALS group P = 00025 P = 00002 and P  00001 respectively As control expression levels for betaactin did not show a significant difference between ALS and control groups P = 02118 Our data show that a reduction in electron transport proteins namely FAD synthetase RFK CYC1 and SDHB is seen in patients with ALS It is possible that this may have an effect on oxygendependent metabolic pathways Human motor neurons may be particularly susceptible to injury if there is suboptimal oxidative metabolism

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