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Gene Review

Pdha1  -  pyruvate dehydrogenase E1 alpha 1

Mus musculus

Synonyms: PDHE1-A type I, Pdha-1, Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
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High impact information on Pdha1


Biological context of Pdha1

  • Exclusion of three candidate genes, Grpr, Cxn33, and Pdha1, for the X-linked cataract gene on the distal region of the mouse chromosome X [6].
  • Two conserved loci, DXHX674h and DXHX679h, which map to Xp11.22-Xp11.21 on the human X chromosome short arm, have been positioned between the loci for proteolipid protein (Plp) and the E1a subunit of pyruvate dehydrogenase (Pdha1) in the distal region of the mouse X chromosome using Mus musculus x Mus spretus interspecific backcrosses [7].
  • Homozygous Pdha1(flox8) females were bred with male mice carrying a wild-type Pdha1 allele and a transgene that ubiquitously expresses the Cre recombinase to produce progeny with a deletion in exon 8, Pdha1(Deltaex8) [8].
  • Inactivation of the murine pyruvate dehydrogenase (Pdha1) gene and its effect on early embryonic development [8].
  • Comparison of the predicted amino acid sequence with those of the somatic and testis-specific E1 alpha forms of man and mouse and the somatic E1 alpha form of rat indicates the change of a serine residue, believed to be phosphorylated in vivo by pyruvate dehydrogenase E1 alpha-specific kinase, to an alanine at position 233 [9].

Anatomical context of Pdha1

  • Developmental expression of CAT activity in the testes was similar to that of the endogenous Pdha-1 subunit expression in the testes [10].
  • We examined whether the expression of PDHE1alpha mRNA is altered by DHA treatment in differentiated PC12 cells [11].

Associations of Pdha1 with chemical compounds

  • Using gene targeting technology, a silent mutation was introduced into the murine X-linked Pdha1 gene that encodes the alpha subunit of the pyruvate dehydrogenase or E1 component of the complex [8].
  • PDHE1alpha mRNA was reduced by supplementation of DHA with a significant decrease in cellular ATP level [11].

Other interactions of Pdha1


  1. A novel X gene with a widely transcribed Y-linked homologue escapes X-inactivation in mouse and human. Agulnik, A.I., Mitchell, M.J., Mattei, M.G., Borsani, G., Avner, P.A., Lerner, J.L., Bishop, C.E. Hum. Mol. Genet. (1994) [Pubmed]
  2. The expression pattern of the pyruvate dehydrogenase E1 alpha subunit genes during spermatogenesis in adult mouse. Takakubo, F., Dahl, H.H. Exp. Cell Res. (1992) [Pubmed]
  3. An integrated genetic and man-mouse comparative map of the DXHXS674-Pdha1 region of the mouse X chromosome. Blair, H.J., Uwechue, I.C., Barsh, G.S., Rowe, P.S., Boyd, Y. Genomics (1998) [Pubmed]
  4. Expression of X-linked genes in androgenetic, gynogenetic, and normal mouse preimplantation embryos. Latham, K.E., Rambhatla, L. Dev. Genet. (1995) [Pubmed]
  5. Comparative mapping of the Grpr locus on the X chromosomes of man and mouse. Maslen, G.L., Boyd, Y. Genomics (1993) [Pubmed]
  6. Exclusion of three candidate genes, Grpr, Cxn33, and Pdha1, for the X-linked cataract gene on the distal region of the mouse chromosome X. Zhou, E., Favor, J., Silvers, W., Stambolian, D. Mamm. Genome (1995) [Pubmed]
  7. New insights into the man-mouse comparative map of the X chromosome. Blair, H.J., Reed, V., Laval, S.H., Boyd, Y. Genomics (1994) [Pubmed]
  8. Inactivation of the murine pyruvate dehydrogenase (Pdha1) gene and its effect on early embryonic development. Johnson, M.T., Mahmood, S., Hyatt, S.L., Yang, H.S., Soloway, P.D., Hanson, R.W., Patel, M.S. Mol. Genet. Metab. (2001) [Pubmed]
  9. Characterization of cDNAs encoding the rat testis-specific E1 alpha subunit of the pyruvate dehydrogenase complex: comparison of expression of the corresponding mRNA with that of the somatic E1 alpha subunit. Cullingford, T.E., Clark, J.B., Phillips, I.R. Biochim. Biophys. Acta (1993) [Pubmed]
  10. Tissue-specific expression of the human pyruvate dehydrogenase alpha (Pdha-1)/chloramphenicol acetyltransferase fusion gene in transgenic mice. Dey, R., Naik, S., Patel, M.S. Biochim. Biophys. Acta (1996) [Pubmed]
  11. Short-term feeding of fish oil down-regulates the expression of pyruvate dehydrogenase E1 alpha subunit mRNA in mouse brain. Nogusa, Y., Yanaka, N., Sumiyoshi, N., Kaseda, Y., Tsuboyama-Kasaoka, N., Ezaki, O., Kato, N. Biosci. Biotechnol. Biochem. (2005) [Pubmed]
  12. Differential expression of two testis-specific transcripts of the mouse Pdha-2 gene during spermatogenesis. Fitzgerald, J., Dahl, H.H., Iannello, R.C. DNA Cell Biol. (1994) [Pubmed]
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