Abstract
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.
Original language | English (US) |
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Pages (from-to) | 305-309 |
Number of pages | 5 |
Journal | FEBS Letters |
Volume | 262 |
Issue number | 2 |
DOIs | |
State | Published - Mar 26 1990 |
Keywords
- (Human liver)
- Amino acid sequence
- Branched-chain E1b-β
- Nucleotide sequence
- cDNA
ASJC Scopus subject areas
- Biophysics
- Structural Biology
- Biochemistry
- Molecular Biology
- Genetics
- Cell Biology