PDHX antibody
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Name
PDHX antibody
Category
Primary Antibodies
Provider
FineTest
Reference
FNab06271
Tested Applications
ELISA, IHC, IP, WB
Description
Required for anchoring dihydrolipoamide dehydrogenase(E3) to the dihydrolipoamide transacetylase(E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
Documentos del producto
Especificaciones del producto
| Category | Primary Antibodies |
| Immunogen Target | pyruvate dehydrogenase complex, component X (PDHX) |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Recommended Dilution | WB: 1:500-1:2000; IP: 1:200-1:1000; IHC: 1:20-1:200 |
| Clonality | polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Observed MW | 54kd |
| Purity | ≥95% as determined by SDS-PAGE |
| Purification | Immunogen affinity purified |
| Size 1 | 100µg |
| Form | liquid |
| Tested Applications | ELISA, IHC, IP, WB |
| Storage | PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20℃ for 12 months(Avoid repeated freeze / thaw cycles.) |
| UniProt ID | O00330 |
| Gene ID | 8050 |
| Alias | DLDBP, E3BP, OPDX, PDX1, proX, pyruvate dehydrogenase complex component X, PDHXD |
| Background | Antibody anti-PDHX |
| Status | RUO |
| Note | Mol. Weight 54 kDa |
Background
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.
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