Abstract
The cDNAs encoding the predicted precursor proteins of human pyruvate dehydrogenase α and β subunits (PDH α and β) have been cloned and sequenced. The amino acid sequences of PDH α and β deduced from their nucleotide sequences were highly homologous with those of porcine PDH. The phosphorylation sites and TPP-binding motif were also conserved in human PDH α. Human PDH α and β genes have been cloned using radio-labeled PDH α and β cDNAs. The PDH α gene spans 17.08 kilobase paris (kb) and is composed of 11 exons and 10 introns. The 18 kb clone of PDH β gene contained about major coding region of 9 kb at its 3'-flanking region. The PDH β gene is composed of 10 exons and 9 introns. All intro/exon splice junctions of two genes follow the GT/AG rule. The 5'-flanking regions of two genes contain typical consensus promoter sequence. Primer extension analysis indicated that the transcription start sites of the PDH α and β genes and thymine and adenine residues located 124 and 132 bases upstream from initiation codon in exon 1, respectively.