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Biochemistry of glutaric aciduria type I: activities of in vitro expressed wild-type and mutant cDNA encoding human glutaryl-CoA dehydrogenase.

作者信息

Liesert M, Zschocke J, Hoffmann G F, Mühlhäuser N, Buckel W

机构信息

Department of Biology, Philipps University Marburg, Germany.

出版信息

J Inherit Metab Dis. 1999 May;22(3):256-8. doi: 10.1023/a:1005525903207.

DOI:10.1023/a:1005525903207
PMID:10384381
Abstract
摘要

相似文献

1
Biochemistry of glutaric aciduria type I: activities of in vitro expressed wild-type and mutant cDNA encoding human glutaryl-CoA dehydrogenase.I型戊二酸血症的生物化学：体外表达的编码人戊二酰辅酶A脱氢酶的野生型和突变型cDNA的活性
J Inherit Metab Dis. 1999 May;22(3):256-8. doi: 10.1023/a:1005525903207.
2
[Glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I)].[戊二酰辅酶A脱氢酶缺乏症（I型戊二酸尿症）]
Ryoikibetsu Shokogun Shirizu. 1998(18 Pt 1):332-5.
3
Conservation of central nervous system glutaryl-coenzyme A dehydrogenase in fruit-eating bats with glutaric aciduria and deficient hepatic glutaryl-coenzyme A dehydrogenase.食果蝙蝠中枢神经系统戊二酰辅酶A脱氢酶的保存情况，这些蝙蝠患有戊二酸尿症且肝脏戊二酰辅酶A脱氢酶缺乏。
J Biol Chem. 1988 Nov 25;263(33):17258-61.
4
Glutaric aciduria type I with high residual glutaryl-CoA dehydrogenase activity.I型戊二酸尿症伴高残留戊二酰辅酶A脱氢酶活性
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5
Monitoring response to therapy in glutaric aciduria by liquid chromatography.
Clin Chem. 1986 Jun;32(6):1231.
6
CT scans of infants with glutaric aciduria.患有戊二酸尿症的婴儿的CT扫描
Dev Med Child Neurol. 1985 Jun;27(3):403-4. doi: 10.1111/j.1469-8749.1985.tb04559.x.
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Subdural hematomas and glutaric aciduria type I.硬膜下血肿与I型戊二酸尿症
Pediatrics. 2001 Feb;107(2):451. doi: 10.1542/peds.107.2.451.
8
Glutaric aciduria type 1 in adulthood.成人型1型戊二酸血症
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Glutaric aciduria type II: evidence for a defect related to the electron transfer flavoprotein or its dehydrogenase.II型戊二酸尿症：与电子传递黄素蛋白或其脱氢酶相关缺陷的证据。
Pediatr Res. 1984 Jul;18(7):663-7. doi: 10.1203/00006450-198407000-00020.
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Glutaric aciduria type I: unusual biochemical presentation.
J Pediatr. 1992 Jul;121(1):83-6. doi: 10.1016/s0022-3476(05)82548-x.

引用本文的文献

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Glutaryl-CoA Dehydrogenase Misfolding in Glutaric Acidemia Type 1.1 型戊二酸血症中谷氨酰辅酶 A 脱氢酶的错误折叠
Int J Mol Sci. 2023 Aug 24;24(17):13158. doi: 10.3390/ijms241713158.
2
Challenges for basic research in glutaryl-CoA dehydrogenase deficiency.戊二酰辅酶A脱氢酶缺乏症基础研究面临的挑战。
J Inherit Metab Dis. 2004;27(6):843-9. doi: 10.1023/B:BOLI.0000045768.38073.22.
3
Mutation analysis in glutaric aciduria type I.I型戊二酸血症的突变分析

本文引用的文献

1
Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish.戊二酰辅酶A脱氢酶的基因结构与突变：A421V替代导致酶亚基结合受损，从而在阿米什人群中引发I型戊二酸血症。
Am J Hum Genet. 1996 Nov;59(5):1006-11.
2
Cloning of glutaryl-CoA dehydrogenase cDNA, and expression of wild type and mutant enzymes in Escherichia coli.戊二酰辅酶A脱氢酶cDNA的克隆以及野生型和突变型酶在大肠杆菌中的表达。
Hum Mol Genet. 1995 Sep;4(9):1493-8. doi: 10.1093/hmg/4.9.1493.
3
Purification of glutaryl-CoA dehydrogenase from Pseudomonas sp., an enzyme involved in the anaerobic degradation of benzoate.
J Med Genet. 2000 Mar;37(3):177-81. doi: 10.1136/jmg.37.3.177.
4
Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I.通过稳定同位素稀释分析法测定游离和总戊二酸以及3-羟基戊二酸用于诊断I型戊二酸血症的敏感性和特异性。
J Inherit Metab Dis. 1999 Dec;22(8):867-81. doi: 10.1023/a:1005683222187.
5
Glutaric aciduria type I: from clinical, biochemical and molecular diversity to successful therapy.I型戊二酸尿症：从临床、生化及分子多样性到成功治疗
J Inherit Metab Dis. 1999 Jun;22(4):381-91. doi: 10.1023/a:1005543904484.
从假单胞菌属中纯化戊二酰辅酶A脱氢酶，该酶参与苯甲酸的厌氧降解。
Arch Microbiol. 1993;159(2):174-81. doi: 10.1007/BF00250279.
4
Mechanism of action of glutaryl-CoA and butyryl-CoA dehydrogenases. Purification of glutaryl-CoA dehydrogenase.戊二酰辅酶A和丁酰辅酶A脱氢酶的作用机制。戊二酰辅酶A脱氢酶的纯化。
Biochemistry. 1981 Mar 17;20(6):1481-90. doi: 10.1021/bi00509a012.
5
Mammalian metabolism of glutaric acid.
J Biol Chem. 1969 Mar 25;244(6):1461-7.
6
An acyl-coenzyme A dehydrogenase assay utilizing the ferricenium ion.一种利用铁离子的酰基辅酶A脱氢酶测定法。
Anal Biochem. 1990 May 1;186(2):280-4. doi: 10.1016/0003-2697(90)90080-s.
7
Glutaric aciduria; a "new" disorder of amino acid metabolism.戊二酸尿症；一种氨基酸代谢的“新”紊乱症。
Biochem Med. 1975 Jan;12(1):12-21. doi: 10.1016/0006-2944(75)90091-5.