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

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

相似文献

1
Bifunctional protein deficiency: complementation within the same group suggesting differential enzyme defects and clues to the underlying basis.
J Inherit Metab Dis. 1998 Jun;21(3):298-301. doi: 10.1023/a:1005396912015.
2
D-hydroxyacyl-CoA dehydrogenase deficiency. Identification of a new peroxisomal disorder with implications for other disorders of beta-oxidation.D-羟酰基辅酶A脱氢酶缺乏症。一种新的过氧化物酶体疾病的鉴定及其对其他β-氧化障碍疾病的意义。
Adv Exp Med Biol. 1999;466:365-9.
3
Peroxisomal bifunctional protein from rat liver is a trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities.大鼠肝脏中的过氧化物酶体双功能蛋白是一种具有2-烯酰辅酶A水合酶、3-羟酰辅酶A脱氢酶和δ3,δ2-烯酰辅酶A异构酶活性的三功能酶。
J Biol Chem. 1990 Feb 15;265(5):2446-9.
4
Substrate stereospecificities of rat liver peroxisomal 3-hydroxyacyl-CoA dehydrogenases.大鼠肝脏过氧化物酶体3-羟基酰基辅酶A脱氢酶的底物立体特异性
Ann N Y Acad Sci. 1996 Dec 27;804:680-1. doi: 10.1111/j.1749-6632.1996.tb18665.x.
5
Bifunctional enzyme deficiency: identification of a new type of peroxisomal disorder in a patient with an impairment in peroxisomal beta-oxidation of unknown aetiology by means of complementation analysis.双功能酶缺乏症：通过互补分析在一名病因不明的过氧化物酶体β氧化受损患者中鉴定出一种新型过氧化物酶体疾病。
J Inherit Metab Dis. 1992;15(3):385-8. doi: 10.1007/BF02435983.
6
Developmental and pathological expression of peroxisomal enzymes: their relationship of D-bifunctional protein deficiency and Zellweger syndrome.过氧化物酶体酶的发育和病理表达：它们与D-双功能蛋白缺乏症和泽尔韦格综合征的关系。
Brain Res. 2000 Mar 6;858(1):40-7. doi: 10.1016/s0006-8993(99)02423-3.
7
Complementation analysis of fibroblasts from peroxisomal fatty acid oxidation deficient patients shows high frequency of bifunctional enzyme deficiency plus intragenic complementation: unequivocal evidence for differential defects in the same enzyme protein.对过氧化物酶体脂肪酸氧化缺陷患者的成纤维细胞进行互补分析，结果显示双功能酶缺陷和基因内互补的频率很高：这是同一酶蛋白存在不同缺陷的确凿证据。
Biochem Biophys Res Commun. 1997 Jun 9;235(1):176-9. doi: 10.1006/bbrc.1997.6755.
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Regulation of rat hepatic peroxisomal enoyl-CoA hydratase-3-hydroxyacyl-CoA dehydrogenase bifunctional enzyme by thyroid hormone.
Biochem Biophys Res Commun. 1992 May 29;185(1):211-6. doi: 10.1016/s0006-291x(05)80977-5.
9
Peroxisomal disorders: overview.过氧化物酶体疾病：概述
Ann N Y Acad Sci. 1996 Dec 27;804:427-41. doi: 10.1111/j.1749-6632.1996.tb18634.x.
10
Peroxisomal bifunctional enzyme deficiency with associated retinal findings.伴有相关视网膜表现的过氧化物酶体双功能酶缺乏症。
Ophthalmic Genet. 1997 Jun;18(2):93-9. doi: 10.3109/13816819709057121.

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Modelling Peroxisomal Disorders in Zebrafish.斑马鱼中过氧化物酶体疾病的建模
Cells. 2025 Jan 20;14(2):147. doi: 10.3390/cells14020147.

本文引用的文献

1
Genetic heterogeneity in patients with a disorder of peroxisomal beta-oxidation: a complementation study based on pristanic acid beta-oxidation suggesting different enzyme defects.过氧化物酶体β-氧化紊乱患者的遗传异质性：基于降植烷酸β-氧化的互补研究提示不同的酶缺陷
J Inherit Metab Dis. 1997 Jul;20(3):437-40. doi: 10.1023/a:1005323221660.
2
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.
3
Complementation analysis of fibroblasts from peroxisomal fatty acid oxidation deficient patients shows high frequency of bifunctional enzyme deficiency plus intragenic complementation: unequivocal evidence for differential defects in the same enzyme protein.对过氧化物酶体脂肪酸氧化缺陷患者的成纤维细胞进行互补分析，结果显示双功能酶缺陷和基因内互补的频率很高：这是同一酶蛋白存在不同缺陷的确凿证据。
Biochem Biophys Res Commun. 1997 Jun 9;235(1):176-9. doi: 10.1006/bbrc.1997.6755.
4
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.
5
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.
6
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.
7
Peroxisomal disorders: a review.过氧化物酶体疾病：综述
J Neuropathol Exp Neurol. 1995 Sep;54(5):726-39. doi: 10.1097/00005072-199509000-00016.
8
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.
9
Peroxisomal bifunctional enzyme deficiency.过氧化物酶体双功能酶缺乏症
J Clin Invest. 1989 Mar;83(3):771-7. doi: 10.1172/JCI113956.
10
Plasma bile acids in patients with peroxisomal dysfunction syndromes: analysis by capillary gas chromatography-mass spectrometry.过氧化物酶体功能障碍综合征患者的血浆胆汁酸：毛细管气相色谱-质谱分析
Eur J Pediatr. 1987 Mar;146(2):166-73. doi: 10.1007/BF02343226.