PDHA1 Proteins, Antibodies, cDNA Clones Research Reagents

PDHA1 (Pyruvate Dehydrogenase E1 Subunit Alpha 1) is a protein coding gene located on human chromosome Xp22.12. PDHA1 is also known as PDHA, PDHAD, PHE1A and PDHCE1A. The human PDHA1 gene encodes a 43296 Da protein containing 390 amino acids. The PDHA1 protein is ubiquitously expressed in heart, fat and other tissues. Among its related pathways are Glucose metabolism and Pyruvate metabolism and Citric Acid (TCA) cycle. PDHA1 is related to oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor and pyruvate dehydrogenase activity. PDHA2 is an important paralog of PDHA1 gene. PDHA1 is associated with some diseases, including Pyruvate Dehydrogenase E1-Alpha Deficiency and Leigh Syndrome With Leukodystrophy.

PDHA1 Protein (2)

    PDHA1 Antibody (1)

      PDHA1 cDNA Clone (20)


      クローニングベクター cDNA 製品

      In expression vector


      クローニングベクター cDNA 製品

      In lentiviral vector


      クローニングベクター cDNA 製品

      PDHA1 qPCR Primer (1)

      PDHA1 Lysate (2)

        PDHA1 の背景知識

        PDHA1, also known as C54G1, is an alpha subunit of pyruvate dehydrogenase. Pyruvate dehydrogenase, together with dihydrolipoamide acetyltransferase and lipoamide dehydrogenase, composes the pyruvate dehydrogenase (PDH) complex. The PDH complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. PDHA1 plays a key role in the function of the PDH complex. Defects in PDHA1 can cause pyruvate dehydrogenase E1-alpha deficiency. Defects in PDHA1 also are the cause of X-linked Leigh syndrome (X-LS). X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord.

        PDHA1 の参考文献

        • Quintana E. et al., 2010, Clin Genet. 77 (5): 474-82.
        • Ah Mew N. et al., 2011, Pediatr Neurol. 45 (1): 57-9.
        • Pinheiro A. et al., 2012, Gene. 506 (1): 173-8.

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