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PDHX is a component of the pyruvate dehydrogenase complex (PDC) located in the mitochondrial matrix, where it plays a critical role in linking glycolysis to the tricarboxylic acid (TCA) cycle by catalyzing the conversion of pyruvate to acetyl-CoA. PDHX functions as the E3-binding protein, anchoring dihydrolipoamide dehydrogenase (E3) to the PDC core and facilitating the transfer of reducing equivalents between PDC subunits. It is essential for the stability and activity of the PDC, ensuring efficient energy production and metabolic homeostasis. Mutations in PDHX are associated with pyruvate dehydrogenase deficiency, a condition characterized by lactic acidosis, developmental delay, and neurological dysfunction due to impaired mitochondrial energy metabolism. PDHX is expressed ubiquitously in energy-demanding tissues such as the brain, muscle, and liver, where it supports ATP production and metabolic adaptation. Knockout studies demonstrate reduced PDC activity, metabolic imbalances, and severe defects in energy-dependent processes, underscoring its importance in cellular respiration and energy metabolism.
Proteins and Peptides
Human
E.Coli
54-501
E.Coli
Lyophilized from a 0.2um filtered solution in PBS with 5% trehalose, pH7.4
Western Blot,ELISA
50μg
200μg
1mg
49.2 kDa
Recombinant
Greater than 95% by SDS-PAGE gel analyses
His tag
Reconstitute with Sterile distilled water
-20°C for 12 months as lyophilized;2-8°C for 1 month under sterile conditions after reconstitution
PDHX
3-4 weeks
DLDBP, E3BP, OPDX, PDX1, proX, pyruvate dehydrogenase complex component X, PDHXD
This product is for research use only.
Required for anchoring dihydrolipoamide dehydrogenase(E3) to the dihydrolipoamide transacetylase(E2) core of the pyruvat...
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Ver másPDHX Antibody is a Rabbit Polyclonal antibody against PDHX. The pyruvate dehydrogenase (PDH) complex is located in the m...
175€ (20 µl)
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