Molecular cloning of the mature E1b‐β subunit of human branched‐chain α‐keto acid dehydrogenase complex

FEBS Letters

Chuang, David T.¹  Chuang, Jacinta L.¹  Cox, Rody P.¹ 

[1] Departments of Biochemistry and Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75235, USA

关键词: cDNA;    Branched-chain E1b-β;    Nucleotide sequence;    Amino acid sequence;    (Human liver);    E1b;    branched-chain α-keto acid decarboxylase;    E1p;    pyruvate dehydrogenase;    E2b;    dihydrolipoyl transacylase;    E3;    dihydrolipoyl dehydrogenase;    SDS;    sodium dodecyl sulfate;    MSUD;    maple syrup urine disease;    bp;    base pairs;    kb;    kilobase pairs;   

DOI  :  10.1016/0014-5793(90)80215-5

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

We have isolated a cDNA encoding the E1b-β subunit of the human branched-chain α-keto acid dehydrogenase complex. The human E1b-β cDNA is 1401 base pairs in length. It encodes the entire mature E1b-β subunit consisting of 342 amino acid residues, and a mitochondrial targeting presequence of 31 residues. The calculated molecular mass of the mature human E1b-β subunit is 37 851 Da, and the calculated isoelectric point is pH 5.18. A hydropathy plot shows that the human E1b-β subunit is highly hydrophobic. Northern blot analysis shows that the human E1b-β mRNA is approximately 1.4 kb in size. It is present at the normal level in fibroblasts from two unrelated maple syrup urine disease patients.

【 授权许可】

Unknown