• 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

完整的α亚基对于人线粒体三功能β氧化蛋白的膜结合是必需的,但对于赋予β亚基3-酮酰基辅酶A硫解酶活性并非必要。

Intact alpha-subunit is required for membrane-binding of human mitochondrial trifunctional beta-oxidation protein, but is not necessary for conferring 3-ketoacyl-CoA thiolase activity to the beta-subunit.

作者信息

Weinberger M J, Rinaldo P, Strauss A W, Bennett M J

机构信息

Department of Pathology, University of Texas Southwestern Medical Center at Dallas 75235, USA.

出版信息

Biochem Biophys Res Commun. 1995 Apr 6;209(1):47-52. doi: 10.1006/bbrc.1995.1468.

DOI:10.1006/bbrc.1995.1468
PMID:7726862
Abstract

We have studied the activities of alpha and beta subunit enzymes of the beta-oxidation trifunctional protein complex in a patient who does not process the alpha-subunit. Long-chain 3-ketoacyl-CoA thiolase, the beta-subunit enzyme, was transported into the mitochondrial matrix, where it expressed normal levels of activity, but was not translocated to the membrane. Thus, intact alpha-subunit is required for trifunctional protein membrane translocation, but is not necessary for conferring activity of the beta-subunit.

摘要

我们研究了一位无法处理α亚基的患者体内β氧化三功能蛋白复合体的α和β亚基酶的活性。β亚基酶长链3-酮脂酰辅酶A硫解酶被转运到线粒体基质中,在那里它表现出正常水平的活性,但没有转运到膜上。因此,完整的α亚基是三功能蛋白膜转运所必需的,但对于赋予β亚基活性并非必要。

相似文献

1
Intact alpha-subunit is required for membrane-binding of human mitochondrial trifunctional beta-oxidation protein, but is not necessary for conferring 3-ketoacyl-CoA thiolase activity to the beta-subunit.完整的α亚基对于人线粒体三功能β氧化蛋白的膜结合是必需的,但对于赋予β亚基3-酮酰基辅酶A硫解酶活性并非必要。
Biochem Biophys Res Commun. 1995 Apr 6;209(1):47-52. doi: 10.1006/bbrc.1995.1468.
2
General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover.由于α亚基或β亚基突变导致的一般线粒体三功能蛋白(TFP)缺乏表现出相似的表型,因为任一亚基的突变都会改变TFP复合物的表达和亚基周转。
Pediatr Res. 2004 Feb;55(2):190-6. doi: 10.1203/01.PDR.0000103931.80055.06. Epub 2003 Nov 19.
3
beta-Oxidation enzymes in fibroblasts from patients with 3-hydroxydicarboxylic aciduria.3-羟基二羧酸尿症患者成纤维细胞中的β-氧化酶
Pediatr Res. 1994 Jul;36(1 Pt 1):111-4. doi: 10.1203/00006450-199407001-00020.
4
Mitochondrial trifunctional protein deficiency. Catalytic heterogeneity of the mutant enzyme in two patients.线粒体三功能蛋白缺乏症。两名患者中突变酶的催化异质性。
J Clin Invest. 1994 Apr;93(4):1740-7. doi: 10.1172/JCI117158.
5
Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.人眼和大脑中的线粒体脂肪酸β-氧化:对长链3-羟基酰基辅酶A脱氢酶缺乏症视网膜病变的影响。
Pediatr Res. 2004 Nov;56(5):744-50. doi: 10.1203/01.PDR.0000141967.52759.83. Epub 2004 Sep 3.
6
A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women.胎儿脂肪酸氧化障碍作为孕妇肝病的一个病因
N Engl J Med. 1999 Jun 3;340(22):1723-31. doi: 10.1056/NEJM199906033402204.
7
Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. II. Purification and properties of enoyl-coenzyme A (CoA) hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein.大鼠肝脏线粒体中的新型脂肪酸β-氧化酶。II. 烯酰辅酶A水合酶/3-羟酰基辅酶A脱氢酶/3-酮酰基辅酶A硫解酶三功能蛋白的纯化及特性
J Biol Chem. 1992 Jan 15;267(2):1034-41.
8
Structural analysis of cDNAs for subunits of human mitochondrial fatty acid beta-oxidation trifunctional protein.人线粒体脂肪酸β-氧化三功能蛋白亚基cDNA的结构分析
Biochem Biophys Res Commun. 1994 Mar 15;199(2):818-25. doi: 10.1006/bbrc.1994.1302.
9
Structural and functional characterization of the recombinant human mitochondrial trifunctional protein.重组人线粒体三功能蛋白的结构与功能特征。
Biochemistry. 2010 Oct 5;49(39):8608-17. doi: 10.1021/bi100742w. Epub 2010 Sep 8.
10
4-bromotiglic acid, a novel inhibitor of thiolases and a tool for assessing the cooperation between the membrane-bound and soluble beta-oxidation systems of rat liver mitochondria.4-溴惕各酸,一种新型硫解酶抑制剂及评估大鼠肝线粒体膜结合和可溶性β-氧化系统之间协同作用的工具。
Biochemistry. 1998 Nov 3;37(44):15548-54. doi: 10.1021/bi981613f.

引用本文的文献

1
Silencing of Mitochondrial Trifunctional Protein A Subunit (HADHA) Increases Lipid Stores, and Reduces Oviposition and Flight Capacity in the Vector Insect .线粒体三功能蛋白A亚基(HADHA)的沉默增加了载体昆虫的脂质储存,并降低了其产卵量和飞行能力。
Front Insect Sci. 2022 Jun 9;2:885172. doi: 10.3389/finsc.2022.885172. eCollection 2022.
2
Integrated small RNA, mRNA and protein omics reveal a miRNA network orchestrating metabolic maturation of the developing human heart.整合的小 RNA、mRNA 和蛋白质组学揭示了一个 miRNA 网络,协调人类心脏发育的代谢成熟。
BMC Genomics. 2023 Nov 23;24(1):709. doi: 10.1186/s12864-023-09801-8.
3
ALKBH7 mediates necrosis via rewiring of glyoxal metabolism.
ALKBH7 通过重排乙二醛代谢介导细胞坏死。
Elife. 2020 Aug 14;9:e58573. doi: 10.7554/eLife.58573.
4
Fatal pitfalls in newborn screening for mitochondrial trifunctional protein (MTP)/long-chain 3-Hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency.新生儿筛查中 mtp/长链 3-羟酰基辅酶 A 脱氢酶(LCHAD)缺陷的致命陷阱。
Orphanet J Rare Dis. 2018 Jul 20;13(1):122. doi: 10.1186/s13023-018-0875-6.
5
Mitochondrial trifunctional protein defects: clinical implications and therapeutic approaches.线粒体三功能蛋白缺陷:临床意义与治疗方法
Adv Drug Deliv Rev. 2008 Oct-Nov;60(13-14):1488-96. doi: 10.1016/j.addr.2008.04.014. Epub 2008 Jul 4.
6
Acute fatty liver of pregnancy: an update on pathogenesis and clinical implications.妊娠期急性脂肪肝:发病机制及临床意义的最新进展
World J Gastroenterol. 2006 Dec 14;12(46):7397-404. doi: 10.3748/wjg.v12.i46.7397.
7
Mammalian mitochondrial beta-oxidation.哺乳动物线粒体β-氧化
Biochem J. 1996 Dec 1;320 ( Pt 2)(Pt 2):345-57. doi: 10.1042/bj3200345.