STRUCTURAL-ANALYSIS OF CDNAS FOR SUBUNITS OF HUMAN MITOCHONDRIAL FATTY-ACID BETA-OXIDATION TRIFUNCTIONAL PROTEIN
STRUCTURAL-ANALYSIS OF CDNAS FOR SUBUNITS OF HUMAN MITOCHONDRIAL FATTY-ACID BETA-OXIDATION TRIFUNCTIONAL PROTEIN
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DOI：
10.1006/bbrc.1994.1302
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发表时间：
1994-03-15
影响因子：
3.1
通讯作者：
HASHIMOTO, T
中科院分区：
文献类型：
--
作者：
KAMIJO, T;AOYAMA, T;HASHIMOTO, T
三功能蛋白缺乏症是一种典型的线粒体长链脂肪酸β-氧化缺陷，是由线粒体长链烯酰辅酶A水合酶/3-羟酰辅酶A脱氢酶/3-酮脂酰辅酶A硫解酶三功能蛋白异常引起的，该三功能蛋白由4摩尔α亚基和4摩尔β亚基组成。我们克隆、测序并表达了人类三功能蛋白的 α 和 β 亚基的以下 cDNA。该 2,690 bp cDNA 克隆具有 2,289 bp 开放阅读框，编码 82,958 Da 前体和 78,969 Da 成熟亚基（α 亚基）。该 cDNA 在哺乳动物细胞中的表达产生了具有长链烯酰基-CoA 水合酶和长链 3-羟酰基-CoA 脱氢酶活性的多肽。该 1,991 bp cDNA 克隆具有 1,422 bp 开放阅读框，编码 51,293 Da 前体和 47,484 Da 成熟亚基（β 亚基）。该 cDNA 在哺乳动物细胞中的表达产生了具有长链 3-酮酰基-CoA 硫解酶活性的多肽。 (C) 1994 年学术出版社
Trifunctional protein deficiency, a typical mitochondrial long-chain fatty acid beta- oxidation defect, is caused by the abnormality of mitochondrial long-chain enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein consisting of four moles of alpha-subunit and four moles of beta-subunit. We cloned, sequenced, and expressed the following cDNAs for the alpha- and beta-subunits of human trifunctional protein. The 2,690-bp cDNA clone had a 2,289-bp open reading frame encoding a 82,958-Da precursor and a 78,969-Da mature subunit (alpha-subunit). Expression of this cDNA in mammalian cells yielded a polypeptide with the long-chain enoyl-CoA hydratase and long-chain 3-hydroxyacyl-CoA dehydrogenase activities. The 1,991-bp cDNA clone had a 1,422-bp open reading frame encoding a 51,293-Da precursor and a 47,484-Da mature subunit (beta-subunit). Expression of this cDNA in mammalian cells yielded a polypeptide with the long-chain 3-ketoacyl-CoA thiolase activity. (C) 1994 Academic Press, Inc.