• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利：CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法，用中文像聊天一样搜索，搜遍4000万医学文献。AI智能推荐，让科研检索更轻松。

立即免费搜索

文件翻译

保留排版，准确专业，支持PDF/Word/PPT等文件格式，支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述，25分钟生成高质量综述，智能提取关键信息，辅助科研写作。

立即免费体验

Branched chain alpha-keto acid metabolism. I. Isolation, purification, and partial characterization of bovine liver alpha-ketoisocaproic:alpha-keto-beta-methylvaleric acid dehydrogenase.

作者信息

Connelly J L, Danner D J, Bowden J A

出版信息

J Biol Chem. 1968 Mar 25;243(6):1198-203.

PMID:5689906
Abstract
摘要

相似文献

1
Branched chain alpha-keto acid metabolism. I. Isolation, purification, and partial characterization of bovine liver alpha-ketoisocaproic:alpha-keto-beta-methylvaleric acid dehydrogenase.支链α-酮酸代谢。I. 牛肝α-酮异己酸：α-酮-β-甲基戊酸脱氢酶的分离、纯化及部分特性鉴定
J Biol Chem. 1968 Mar 25;243(6):1198-203.
2
Heterogeneity in maple syrup urine disease: aspects of cofactor requirement and complementation in cultured fibroblasts.
Clin Genet. 1977 Apr;11(4):277-84. doi: 10.1111/j.1399-0004.1977.tb01313.x.
3
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.
4
Mammalian alpha-keto acid dehydrogenase complexes. IV. Substrate specificities and kinetic properties of the pig heart pyruvate and 2-oxyoglutarate dehydrogenase complexes.哺乳动物α-酮酸脱氢酶复合体。IV. 猪心脏丙酮酸和2-氧代戊二酸脱氢酶复合体的底物特异性及动力学特性
J Biol Chem. 1969 Mar 10;244(5):1183-7.
5
Assays for E1 and E2 components of the branched-chain keto acid dehydrogenase complex.
Methods Enzymol. 1988;166:146-54. doi: 10.1016/s0076-6879(88)66021-6.
6
Further observations on the biochemical lesion in maple syrup urine disease.
Nature. 1967 Apr 15;214(5085):276. doi: 10.1038/214276a0.
7
Immunosuppressive effects of organic acids accumulating in patients with maple syrup urine disease.
J Inherit Metab Dis. 1995;18(2):165-8. doi: 10.1007/BF00711757.
8
Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex.枫糖尿症中积累的α-酮酸可显著降低大鼠大脑皮层突触质膜上钠钾ATP酶的活性。
Metab Brain Dis. 2007 Mar;22(1):77-88. doi: 10.1007/s11011-007-9046-5.
9
Oxidation of branched chain -ketoacids in Streptococcus faecalis and it's dependence on lipoic acid.
Hoppe Seylers Z Physiol Chem. 1972 Jun;353(6):875-82. doi: 10.1515/bchm2.1972.353.1.875.
10
Blood and tissue branched-chain amino and alpha-keto acid concentrations: effect of diet, starvation, and disease.血液和组织中支链氨基酸及α-酮酸浓度：饮食、饥饿和疾病的影响
Am J Clin Nutr. 1981 Feb;34(2):173-83. doi: 10.1093/ajcn/34.2.173.

引用本文的文献

1
Reversible ATP-induced inactivation of branched-chain 2-oxo acid dehydrogenase.ATP诱导的支链2-氧代酸脱氢酶的可逆失活
Biochem J. 1980 Oct 15;192(1):155-63. doi: 10.1042/bj1920155.
2
Purification of a branched-chain keto acid dehydrogenase from Pseudomonas putida.从恶臭假单胞菌中纯化支链酮酸脱氢酶。
J Bacteriol. 1981 Nov;148(2):647-52. doi: 10.1128/jb.148.2.647-652.1981.
3
Enzymatic and pharmacokinetic studies on the metabolism of branched chain alpha-keto acids in the rat.大鼠体内支链α-酮酸代谢的酶学及药代动力学研究。
Z Ernahrungswiss. 1983 Jan;22(1):14-26. doi: 10.1007/BF02020781.
4
Normal 2-aminobutyrate oxidation and increased valine oxidation in fibroblasts deficient in pyruvate dehydrogenase.丙酮酸脱氢酶缺乏的成纤维细胞中正常的2-氨基丁酸氧化和缬氨酸氧化增加
J Inherit Metab Dis. 1982;5(1):55-7. doi: 10.1007/BF01799755.
5
Cytochemical localization by ferricyanide reduction of -hydroxy acid oxidase activity in peroxisomes of rat kidney.用铁氰化物还原法对大鼠肾脏过氧化物酶体中α-羟酸氧化酶活性进行细胞化学定位。
Histochemie. 1971;27(2):137-58. doi: 10.1007/BF00284956.
6
Ketosis.酮症
Trans Am Clin Climatol Assoc. 1973;84:184-202.
7
Differential effects of 2-oxo acids on pyruvate utilization and fatty acid synthesis in rat brain.2-氧代酸对大鼠脑内丙酮酸利用及脂肪酸合成的不同影响
Biochem J. 1974 Apr;140(1):25-9. doi: 10.1042/bj1400025.
8
D- and L-isoleucine metabolism and regulation of their pathways in Pseudomonas putida.恶臭假单胞菌中D-异亮氨酸和L-异亮氨酸的代谢及其途径的调控
J Bacteriol. 1974 Apr;118(1):103-11. doi: 10.1128/jb.118.1.103-111.1974.
9
Regulation of branched-chain amino acid oxidation in isolated muscles, nerves and aortas of rats.大鼠离体肌肉、神经和主动脉中支链氨基酸氧化的调节
Biochem J. 1975 Jun;148(3):363-74. doi: 10.1042/bj1480363.
10
Maple syrup urine disease: branched-chain amino acid concentrations and metabolism in cultured human lymphoblasts.枫糖尿症：培养的人淋巴母细胞中的支链氨基酸浓度及代谢
Biochem Genet. 1976 Aug;14(7-8):527-39. doi: 10.1007/BF00485832.