人支链α-酮酸脱氢酶对硫胺素和硫胺素焦磷酸的体内及体外反应 - Suppr

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超能文献

In vivo and in vitro response of human branched chain alpha-ketoacid dehydrogenase to thiamine and thiamine pyrophosphate.

作者信息

Danner D J, Wheeler F B, Lemmon S K, Elsas L J

出版信息

Pediatr Res. 1978 Mar;12(3):235-8. doi: 10.1203/00006450-197803000-00016.

DOI:10.1203/00006450-197803000-00016

PMID:643394

Abstract

摘要

相似文献

In vivo and in vitro response of human branched chain alpha-ketoacid dehydrogenase to thiamine and thiamine pyrophosphate.人支链α-酮酸脱氢酶对硫胺素和硫胺素焦磷酸的体内及体外反应

Pediatr Res. 1978 Mar;12(3):235-8. doi: 10.1203/00006450-197803000-00016.

Thiamine response in maple syrup urine disease.枫糖尿症中的硫胺素反应。

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The role of thiamin in maple syrup urine disease.硫胺素在枫糖尿症中的作用。

Ann N Y Acad Sci. 1982;378:404-21. doi: 10.1111/j.1749-6632.1982.tb31214.x.

Substrate specificity and stabilization by thiamine pyrophosphate of rat liver branched chain alpha-ketoacid dehydrogenase.大鼠肝脏支链α-酮酸脱氢酶的底物特异性及硫胺素焦磷酸对其的稳定作用

Biochem Med. 1978 Feb;19(1):27-38. doi: 10.1016/0006-2944(78)90004-2.

Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate.硫胺素反应性枫糖尿症：突变型支链α-酮酸脱氢酶对α-酮异戊酸和硫胺素焦磷酸的亲和力降低。

Proc Natl Acad Sci U S A. 1982 May;79(10):3300-4. doi: 10.1073/pnas.79.10.3300.

Stabilization of mammalian liver branched-chain alpha-ketoacid dehydrogenase by thiamin pyrophosphate.硫胺素焦磷酸对哺乳动物肝脏支链α-酮酸脱氢酶的稳定作用。

Arch Biochem Biophys. 1980 Jun;202(1):23-8. doi: 10.1016/0003-9861(80)90401-4.

Sequence of the E1 alpha subunit of branched-chain alpha-ketoacid dehydrogenase in two patients with thiamine-responsive maple syrup urine disease.两名硫胺素反应性枫糖尿症患者支链α-酮酸脱氢酶E1α亚基的序列分析

Am J Hum Genet. 1990 Apr;46(4):843-6.

Reversion of the maple syrup urine disease phenotype of impaired branched chain alpha-ketoacid dehydrogenase complex activity in fibroblasts from an affected child.一名患病儿童成纤维细胞中支链α-酮酸脱氢酶复合体活性受损所致枫糖尿症表型的逆转。

J Biol Chem. 1989 Sep 5;264(25):14597-600.

Maple syrup urine disease in a Bedouin tribe: pre- and postnatal diagnosis.贝都因部落中的枫糖尿症：产前和产后诊断

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Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease.作为肟衍生物分离出的支链α-酮酸：与枫糖尿症中相应的羟基酸和氨基酸的关系。

Metabolism. 1974 Mar;23(3):257-65. doi: 10.1016/0026-0495(74)90064-x.

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Megavitamin therapy in inherited metabolic disorders.遗传性代谢紊乱中的大剂量维生素疗法。

Indian J Pediatr. 1981 Sep-Oct;48(394):635-46. doi: 10.1007/BF02821592.

Proc Natl Acad Sci U S A. 1982 May;79(10):3300-4. doi: 10.1073/pnas.79.10.3300.

Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.异戊酸血症患者成纤维细胞中特异性线粒体异戊酰辅酶A脱氢酶缺乏的证明。

Proc Natl Acad Sci U S A. 1980 Jan;77(1):580-3. doi: 10.1073/pnas.77.1.580.

Decreased transport of ornithine across the inner mitochondrial membrane as a cause of hyperornithinaemia.鸟氨酸跨线粒体内膜转运减少作为高鸟氨酸血症的一个病因。

J Inherit Metab Dis. 1982;5(1):41-7. doi: 10.1007/BF01799753.

Branched-chain ketoacid dehydrogenase activity and growth of normal and mutant human fibroblasts: the effect of branched-chain amino acid concentration in culture medium.支链酮酸脱氢酶活性与正常及突变型人成纤维细胞的生长：培养基中支链氨基酸浓度的影响

Biochem Genet. 1983 Oct;21(9-10):895-905. doi: 10.1007/BF00483948.

Direct physical evidence for stabilization of branched-chain alpha-ketoacid dehydrogenase by thiamin pyrophosphate.硫胺素焦磷酸对支链α-酮酸脱氢酶稳定作用的直接物理证据。

Am J Hum Genet. 1984 Jul;36(4):802-7.

Absence of branched chain acyl-transferase as a cause of maple syrup urine disease.缺乏支链酰基转移酶作为枫糖尿症的病因

J Clin Invest. 1985 Mar;75(3):858-60. doi: 10.1172/JCI111783.

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