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Human PDHA1 / C54G1 Protein (aa 30-390, His & GST Tag)

PDHA,PDHAD,PDHCE1A,PHE1A

Catalog Number P14567-H20B
Organism Species Human
Host Baculovirus-Insect Cells
Synonyms PDHA,PDHAD,PDHCE1A,PHE1A
Molecular Weight The recombinant human PDHA1/GST chimera consists of 598 amino acids and has a calculated molecular mass of 68 kDa. The recombinant protein migrates as an approximately 65 kDa band in SDS-PAGE under reducing conditions.
predicted N Met
SDS-PAGE
Purity > 95 % as determined by SDS-PAGE
Protein Construction A DNA sequence encoding the human PDHA1 (P08559-1) (Phe30-Ser390) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus.
Bio-activity
Research Area Epigenetics |Cell cycle |Markers
Formulation Lyophilized from sterile 20mM Tris, 500mM Nacl, 3mM DTT, 10% gly, pH 8.0.
1. Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.
Background 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.
Reference
  • 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.