Bifunctional protein deficiency: complementation within the same group suggesting differential enzyme defects and clues to the underlying basis.

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作者信息

Van Grunsven E G, van Berkel E, Lemonde H, Clayton P T, Wanders R J

机构信息

University of Amsterdam, Academic Medical Center, Department of Clinical Chemistry, The Netherlands.

出版信息

J Inherit Metab Dis. 1998 Jun;21(3):298-301. doi: 10.1023/a:1005396912015.

DOI:10.1023/a:1005396912015

PMID:9686380

Abstract

摘要

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J Inherit Metab Dis. 1997 Jul;20(3):437-40. doi: 10.1023/a:1005323221660.

Sterol carrier protein X (SCPx) is a peroxisomal branched-chain beta-ketothiolase specifically reacting with 3-oxo-pristanoyl-CoA: a new, unique role for SCPx in branched-chain fatty acid metabolism in peroxisomes.固醇载体蛋白X（SCPx）是一种过氧化物酶体支链β-酮硫解酶，它特异性地与3-氧代-降植烷酰辅酶A反应：SCPx在过氧化物酶体支链脂肪酸代谢中的一种新的独特作用。

Biochem Biophys Res Commun. 1997 Jul 30;236(3):565-9. doi: 10.1006/bbrc.1997.7007.

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Physiological role of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein.D-3-羟酰基辅酶A脱水酶/D-3-羟酰基辅酶A脱氢酶双功能蛋白的生理作用。

J Biochem. 1997 Mar;121(3):506-13. doi: 10.1093/oxfordjournals.jbchem.a021615.

Pre- and postnatal diagnosis of peroxisomal disorders using stable-isotope dilution gas chromatography--mass spectrometry.使用稳定同位素稀释气相色谱 - 质谱法进行过氧化物酶体疾病的产前和产后诊断。

J Inherit Metab Dis. 1995;18 Suppl 1:45-60. doi: 10.1007/BF00711428.

The reactions catalyzed by the inducible bifunctional enzyme of rat liver peroxisomes cannot lead to the formation of bile acids.大鼠肝脏过氧化物酶体的诱导型双功能酶所催化的反应不会导致胆汁酸的形成。

Biochem Biophys Res Commun. 1996 Apr 16;221(2):271-8. doi: 10.1006/bbrc.1996.0585.

Peroxisomal disorders: a review.过氧化物酶体疾病：综述

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Properties of mitochondria and peroxisomal enoyl-CoA hydratases from rat liver.大鼠肝脏线粒体和过氧化物酶体烯酰辅酶A水合酶的特性

J Biochem. 1980 Oct;88(4):1059-70. doi: 10.1093/oxfordjournals.jbchem.a133057.

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