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  Brain pyruvate and 2‐oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid

Bunik, V., Raddatz, G., Wanders, R., & Reiser, G. (2006). Brain pyruvate and 2‐oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid. FEBS Letters, 580(14), 3551-3557. doi:10.1016/j.febslet.2006.05.040.

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Item Permalink: http://hdl.handle.net/21.11116/0000-0004-83CB-3 Version Permalink: http://hdl.handle.net/21.11116/0000-0004-83CC-2
Genre: Journal Article

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 Creators:
Bunik, VI, Author
Raddatz, G1, 2, Author              
Wanders, RJA, Author
Reiser, G, Author
Affiliations:
1Former Department MRZ, Max Planck Institute for Biological Cybernetics, Max Planck Society, ou_2528700              
2Max Planck Institute for Biological Cybernetics, Max Planck Society, Spemannstrasse 38, 72076 Tübingen, DE, ou_1497794              

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 Abstract: Pyruvate and 2‐oxoglutarate dehydrogenase complexes are strongly inhibited by phytanoyl‐CoA (IC50 ≈ 10−6–10−7 M). Palmitoyl‐CoA is 10‐fold less potent. Phytanic or palmitic acids have no inhibitory effect up to 0.3 mM. At the substrate saturation, the acyl‐CoA's affect the first and second enzymatic components of the 2‐oxoglutarate dehydrogenase complex, while the third component is inhibited only at a low saturation with its substrate dihydrolipoamide. Thus, key regulatory branch points of mitochondrial metabolism are targets of a cellular derivative of phytanic acid. Decreased activity of the complexes might therefore contribute to neurological symptoms upon accumulation of phytanic acid in Refsum disease.

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 Dates: 2006-06
 Publication Status: Published in print
 Pages: -
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 Table of Contents: -
 Rev. Method: -
 Identifiers: DOI: 10.1016/j.febslet.2006.05.040
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Title: FEBS Letters
  Other : FEBS Lett.
Source Genre: Journal
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Publ. Info: Amsterdam : Elsevier
Pages: - Volume / Issue: 580 (14) Sequence Number: - Start / End Page: 3551 - 3557 Identifier: ISSN: 0014-5793
CoNE: https://pure.mpg.de/cone/journals/resource/954925399501