Abnormal activation of private dehydrogenase in Leigh disease fibroblasts
- 1 May 1982
- journal article
- research article
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurology
- Vol. 32 (5), 555
- https://doi.org/10.1212/wnl.32.5.555
Abstract
Incubation with dichloroacetate increased activity of the pyruvate dehydrogenase complex (PDHC) in disrupted fibroblasts from controls but not from two patients with autopsy-proved Leigh disease. These results are consistent with a genetically determined aberration of the regulation of PDHC in this disorder, although further studies are necessary to define the aberration.This publication has 5 references indexed in Scilit:
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