{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T16:22:24Z","timestamp":1760977344990},"reference-count":25,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[1997,9,1]],"date-time":"1997-09-01T00:00:00Z","timestamp":873072000000},"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":[[1997,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>It was found that \u03b1\u2010oxidation in rat liver is a peroxisomal process, consisting of an activation, a 2\u2010hydroxylation, and a reaction leading to the production of formate. \u03b1\u2010Oxidation of 3\u2010methyl\u2010substituted fatty acids was quantified by measuring the production of formate and CO2, and the production of a 2\u2010hydroxy\u20103\u2010methylacyl\u2010CoA\u2010intermediate was demonstrated. We wanted to extend these findings to human liver, in view of the controversy over the subcellular localization of \u03b1\u2010oxidation in man.<\/jats:p><jats:p>In homogenates from human liver, rates of \u03b1\u2010oxidation were highest when measured in the presence of ATP, CoA, Mg2+, 2\u2010oxoglutarate, ascorbate and Fe2+. In subcellular fractions prepared by differential centrifugation and in fractions obtained after subfractionation of a peroxisome\u2010enriched fraction on a Percoll gradient, production of formate and of a 2\u2010hydroxy\u20103\u2010methylacyl\u2010CoA intermediate coincided with the peroxisomal marker catalase. In broken fractions, production of CO2 was almost negligible as compared to formate production.<\/jats:p><jats:p>We conclude from our findings that in human liver, as in rat liver, \u03b1\u2010oxidation of 3\u2010methyl\u2010substituted fatty acids is a peroxisomal process, consisting of an activation reaction, a 2\u2010hydroxylation reaction and a reaction or reactions leading to the generation of formate as a primary product which is subsequently converted to CO2. Furthermore activation, 2\u2010hydroxylation and generation of formate appear to be coupled (see Casteels et al 1996). These data demonstrate that Refsum disease should indeed be classified as a peroxisomal disease.<\/jats:p>","DOI":"10.1023\/a:1005370325260","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T21:48:34Z","timestamp":1040507314000},"page":"665-673","source":"Crossref","is-referenced-by-count":12,"title":["Peroxisomal localization of \u03b1\u2010oxidation in human liver"],"prefix":"10.1002","volume":"20","author":[{"given":"M.","family":"Casteels","sequence":"first","affiliation":[{"name":"Katholieke Universiteit Leuven, Afdeling Farmacologie  Campus Gasthuisberg Leuven Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Croes","sequence":"additional","affiliation":[{"name":"Katholieke Universiteit Leuven, Afdeling Farmacologie  Campus Gasthuisberg Leuven Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P. P.","family":"Van Veldhoven","sequence":"additional","affiliation":[{"name":"Katholieke Universiteit Leuven, Afdeling Farmacologie  Campus Gasthuisberg Leuven Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G. P.","family":"Mannaerts","sequence":"additional","affiliation":[{"name":"Katholieke Universiteit Leuven, Afdeling Farmacologie  Campus Gasthuisberg Leuven Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1997,9]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-2952(94)90596-7"},{"issue":"1","key":"e_1_2_1_3_1","first-page":"81","article-title":"\u03b1\u2010Oxidation of 3\u2010methyl\u2010substituted fatty acids in liver of rat and man. Formation of a 2\u2010OH\u20103\u2010methylacyl\u2010CoA intermediate","volume":"19","author":"Casteels M","year":"1996","journal-title":"J Inher Metab Dis"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(94)00209-H"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1996.0674h.x"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)47265-6"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9861(92)90565-E"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(94)90136-8"},{"issue":"1","key":"e_1_2_1_9_1","first-page":"5","article-title":"Phytanoyl\u2010CoA hydroxylase deficiency: discovery of the long\u2010sought enzyme defect in Refsum disease","volume":"19","author":"Jansen GA","year":"1996","journal-title":"J Inher Metab Dis"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1995.545zz.x"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)42022-X"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(93)80395-B"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442211"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2920457"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)43207-9"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)36911-9"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)82160-3"},{"key":"e_1_2_1_18_1","first-page":"1533","volume-title":"The Metabolic Basis of Inherited Disease","author":"Steinberg D","year":"1989"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)41399-9"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(67)90352-X"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/0020-711X(94)90131-7"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)54282-4"},{"key":"e_1_2_1_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)61349-3"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00710285"},{"key":"e_1_2_1_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(93)90239-6"},{"key":"e_1_2_1_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01811700"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1005370325260","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1005370325260","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T06:02:13Z","timestamp":1694757733000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1005370325260"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1997,9]]},"references-count":25,"journal-issue":{"issue":"5","published-print":{"date-parts":[[1997,9]]}},"alternative-id":["10.1023\/A:1005370325260"],"URL":"https:\/\/doi.org\/10.1023\/a:1005370325260","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1997,9]]}}}