{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T20:20:31Z","timestamp":1791145231754,"version":"4.1.0"},"reference-count":13,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[1988,12,12]],"date-time":"1988-12-12T00:00:00Z","timestamp":597888000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1989,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The mitochondrial copper concentrations and cytochrome C oxidase activity of the fibroblasts from the patients with Menkes syndrome were investigated. Both the mitochondrial copper concentrations and cytochrome C oxidase activity of fibroblasts from patients with Menkes syndrome were lower than those of the control fibroblasts. These data indicate that the mitochondria of fibroblasts from patients with Menkes syndrome are in a state of copper deficiency. The activity decline of cytochrome C oxidase, a mitochondrial cuproenzyme, seems to be caused by copper deficiency in the mitochondria.<\/jats:p>","DOI":"10.1007\/bf01802032","type":"journal-article","created":{"date-parts":[[2005,6,27]],"date-time":"2005-06-27T23:58:04Z","timestamp":1119916684000},"page":"386-389","source":"Crossref","is-referenced-by-count":32,"title":["Copper deficiency in the mitochondria of cultured skin fibroblasts from patients with menkes syndrome"],"prefix":"10.1002","volume":"12","author":[{"given":"H.","family":"Kodama","sequence":"first","affiliation":[{"name":"Department of Pediatrics Jichi Medical School  Kawachi\u2010gun, Tochigi\u2010ken 329\u201004 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"Okabe","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Jichi Medical School  Kawachi\u2010gun, Tochigi\u2010ken 329\u201004 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Yanagisawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Jichi Medical School  Kawachi\u2010gun, Tochigi\u2010ken 329\u201004 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y.","family":"Kodama","sequence":"additional","affiliation":[{"name":"Ocean Research Institute Tokyo University  Nakano\u2010ku, Tokyo 164 Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1988,12,12]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00504364"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(78)90284-X"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.179.4078.1140"},{"key":"e_1_2_1_5_1","first-page":"969","article-title":"\u03b1\u2010Glucuronidase deficiency mucopolysaccharidosis: methods for enzymatic diagnosis","volume":"82","author":"Glaser J. 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