PMID: 8943006Nov 26, 1996Paper

Molecular characterization of the human peroxisomal branched-chain acyl-CoA oxidase: cDNA cloning, chromosomal assignment, tissue distribution, and evidence for the absence of the protein in Zellweger syndrome

Proceedings of the National Academy of Sciences of the United States of America
E BaumgartP P van Veldhoven

Abstract

Peroxisomes in human liver contain two distinct acyl-CoA oxidases with different substrate specificities: (i) palmitoyl-CoA oxidase, oxidizing very long straight-chain fatty acids and eicosanoids, and (ii) a branched-chain acyl-CoA oxidase (hBRCACox), involved in the degradation of long branched fatty acids and bile acid intermediates. The accumulation of branched fatty acids and bile acid intermediates leads to severe mental retardation and death of the diseased children. In this study, we report the molecular characterization of the hBRCACox, a prerequisite for studying mutations in patients with a single enzyme deficiency. The composite cDNA sequence of hBRCACox, derived from overlapping clones isolated via immunoscreening and hybridization of human liver cDNA expression libraries, consisted of 2225 bases and contained an open reading frame of 2046 bases, encoding a protein of 681 amino acids with a calculated molecular mass of 76,739 Da. The C-terminal tripeptide of the protein is SKL, a known peroxisome targeting signal. Sequence comparison with the other acyl-CoA oxidases and evolutionary analysis revealed that, despite its broader substrate specificity, the hBRCACox is the human homolog of rat trihydroxycoprostanoyl-CoA ...Continue Reading

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Citations

Jul 31, 2008·Histochemistry and Cell Biology·Srikanth Karnati, Eveline Baumgart-Vogt
Feb 17, 2009·Histochemistry and Cell Biology·H Dariush Fahimi
Nov 25, 1998·Molecular Aspects of Medicine·R J Wanders, J M Tager
Oct 10, 2001·Progress in Lipid Research·N M Verhoeven, C Jakobs
Apr 13, 2000·Biochimica Et Biophysica Acta·J K Hiltunen, Y Qin
Dec 10, 2009·The Journal of Biological Chemistry·Michael OgundareWilliam J Griffiths
Dec 5, 2009·Antioxidants & Redox Signaling·Vasily D AntonenkovJ Kalervo Hiltunen
Jun 8, 2006·Annual Review of Biochemistry·Ronald J A Wanders, Hans R Waterham
Aug 16, 2008·PLoS Genetics·Pauline C NgJ Craig Venter
May 28, 2014·Journal of the International Society of Sports Nutrition·Eric R HelmsPeter J Fitschen
Sep 7, 2007·PLoS Biology·Samuel LevyJ Craig Venter
Apr 3, 2012·Biochimica Et Biophysica Acta·Mary C HuntStefan E H Alexson
Sep 21, 2016·Proceedings of the National Academy of Sciences of the United States of America·Sílvia VilarinhoRichard P Lifton
Nov 13, 2012·Molecular Genetics and Metabolism·Kaitlyn KormanikAl-Walid Mohsen
Jul 25, 2017·International Journal of Molecular Sciences·Pierre AndreolettiStéphane Savary
Mar 18, 2008·Hepatology Research : the Official Journal of the Japan Society of Hepatology·Cunshuan XuShaowei Qin
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Aug 31, 2006·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Mary C HuntStefan E H Alexson
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Jun 7, 2021·Annals of Anatomy = Anatomischer Anzeiger : Official Organ of the Anatomische Gesellschaft·Christoph WatermannSrikanth Karnati

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