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Molecular cloning of the mature E1b‐β subunit of human branched‐chain α‐keto acid dehydrogenase complex
Author(s) -
Chuang Jacinta L.,
Cox Rody P.,
Chuang David T.
Publication year - 1990
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/0014-5793(90)80215-5
Subject(s) - complementary dna , protein subunit , maple syrup urine disease , biochemistry , isoelectric point , microbiology and biotechnology , molecular mass , amino acid , biology , molecular cloning , chemistry , gene , enzyme , leucine
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.