{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,16]],"date-time":"2026-03-16T10:58:29Z","timestamp":1773658709256,"version":"3.50.1"},"reference-count":42,"publisher":"Wiley","issue":"14","license":[{"start":{"date-parts":[[2006,5,24]],"date-time":"2006-05-24T00:00:00Z","timestamp":1148428800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FEBS Letters"],"published-print":{"date-parts":[[2006,6,12]]},"abstract":"<jats:p>Pyruvate and 2\u2010oxoglutarate dehydrogenase complexes are strongly inhibited by phytanoyl\u2010CoA (IC<jats:sub>50<\/jats:sub>\n\u2248 10<jats:sup>\u22126<\/jats:sup>\u201310<jats:sup>\u22127<\/jats:sup>\nM). Palmitoyl\u2010CoA is 10\u2010fold less potent. Phytanic or palmitic acids have no inhibitory effect up to 0.3 mM. At the substrate saturation, the acyl\u2010CoA's affect the first and second enzymatic components of the 2\u2010oxoglutarate 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.<\/jats:p>","DOI":"10.1016\/j.febslet.2006.05.040","type":"journal-article","created":{"date-parts":[[2006,5,26]],"date-time":"2006-05-26T00:15:23Z","timestamp":1148602523000},"page":"3551-3557","source":"Crossref","is-referenced-by-count":19,"title":["Brain pyruvate and 2\u2010oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid"],"prefix":"10.1002","volume":"580","author":[{"given":"Victoria I.","family":"Bunik","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00fcnter","family":"Raddatz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald J.A.","family":"Wanders","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Georg","family":"Reiser","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,5,24]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-1981(03)00003-9"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20040583"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2004.08.010"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M513198200"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00512a023"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00512a024"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1989.tb14998.x"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432-1033.2002.03204.x"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432-1033.2003.03470.x"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1385\/MN:31:1-3:043"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432-1033.2003.03468.x"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2744(69)90213-7"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)62650-X"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1975.tb02460.x"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1975.tb02461.x"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)42910-4"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj0920010C"},{"key":"e_1_2_6_19_1","first-page":"1126","article-title":"Regulation of &alpha;-ketoglutarate dehydrogenase complex from pigeon breast muscle","volume":"44","author":"Gomazkova V.S.","year":"1979","journal-title":"Biokhimiia (Russian)"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.jbchem.a130805"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)40452-2"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.35.7.1041"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432-1033.2003.03534.x"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/0020-711X(78)90113-1"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1977.tb11506.x"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9861(78)90014-0"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2340233"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1989.tb15026.x"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi0503100"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00066a007"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/elps.1150181505"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcc.20291"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkg520"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2650849"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(93)81472-C"},{"key":"e_1_2_6_36_1","first-page":"1012","article-title":"Inhibition of &alpha;-ketoglutarate dehydrogenase from pigeon breast muscle by the structural analogs of substarte","volume":"62","author":"Bunik V.I.","year":"1997","journal-title":"Biochem. 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