Concepedia

Dichloroacetate stabilizes the mutant E1α subunit in pyruvate dehydrogenase deficiency

Karl Morten, P. Beattie, Garry K. Brown, Paul M. Matthews

Neurology · 1999 · 27 citations · 19 references

DOI

Abstract

Chronic DCA treatment can increase PDH activity in PDH-deficient cell lines harboring mutations that affect E1alpha stability, suggesting a biochemical criterion by which DCA-responsive patients might be selected.

References

19

Signal-induced site-specific phosphorylation targets I kappa B alpha to the ubiquitin-proteasome pathway.

Zhe Chen, Jeremiah Hagler, Vito J. Palombella et al. · Genes & Development · 1995 · 1.3K citations · Full text

, , +16

A Controlled Clinical Trial of Dichloroacetate for Treatment of Lactic Acidosis in Adults

Peter W. Stacpoole, Elizabeth C. Wright, Thomas G. Baumgartner et al. · New England Journal of Medicine · 1992 · 326 citations · Full text

Chaperone-supervised conversion of prion protein to its protease-resistant form

Shubhik DebBurman, Gregory J. Raymond, Byron Caughey et al. · Proceedings of the National Academy of Sciences · 1997 · 233 citations · Full text

Short‐term dichloroacetate treatment improves indices of cerebral metabolism in patients with mitochondrial disorders

Nicola De Stefano, Paul M. Matthews, Bridget Ford et al. · Neurology · 1995 · 166 citations

X-Chromosome localization of the functional gene for the E1α subunit of the human pyruvate dehydrogenase complex

Ruth M. Brown, Hans‐Henrik M. Dahl, Garry K. Brown · Genomics · 1989 · 136 citations

, , +12