Molecular cloning of the mature E1b-beta subunit of human branched-chain alpha-keto acid dehydrogenase complex |
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| Authors: | J L Chuang R P Cox D T Chuang |
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| Affiliation: | Department of Biochemistry, University of Texas Southwestern Medical Center, Dallas 75235. |
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| Abstract: | We have isolated a cDNA encoding the E1b-beta subunit of the human branched-chain alpha-keto acid dehydrogenase complex. The human E1b-beta cDNA is 1401 base pairs in length. It encodes the entire mature E1b-beta subunit consisting of 342 amino acid residues, and a mitochondrial targeting presequence of 31 residues. The calculated molecular mass of the mature human E1b-beta subunit is 37,851 Da, and the calculated isoelectric point is pH 5.18. A hydropathy plot shows that the human E1b-beta subunit is highly hydrophobic. Northern blot analysis shows that the human E1b-beta 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. |
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