- Poly28604 (See other available formats)
- Other Names
- PDHB, Pyruvate Dehydrogenase Beta
- Rabbit Polyclonal IgG
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- Product Citations
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PDHβ is a component of the E1 pyruvate dehydrogenase (PDHE1). Two PDHβ subunits bind to two PDHα subunits to form the heterotetrameric PDHE1, which plays a role in the overall conversion of pyruvate to acetyl-Coa and CO2. Mutations in PDHβ cause one form of pyruvate dehydrogenase deficiency, which is clinically characterized by the buildup of lactic acid in the body, and a variety of neurological problems. Energy deficient young patients with severe PDH deficiency may have altered brain development, leading to epileptogenesis1.Product Details
- Human, Mouse, Rat
- Antibody Type
- Host Species
- Recombinant PDHb (Leu31~Asn341) with N-terminal His-Tag expressed in E.coli.
- 0.01 M PBS, pH7.4, containing 0.02% NaN3, 50% glycerol.
- The antibody was purified by affinity chromatography.
- 0.5 mg/ml
- Storage & Handling
- Store at 4°C for up to 3 months. For longer term storage aliquot into small volumes and store at -20°C.
WB - Quality tested
IHC-P - Validated
- Recommended Usage
Each lot of this antibody is quality control tested by Western blotting. For Western blotting, the suggested use of this reagent is 0.02 - 0.1 µg per ml. For immunohistochemistry on formalin-fixed paraffin-embedded tissue, a concentration range of 1.0 - 5.0 µg/ml is suggested. It is recommended that the reagent be titrated for optimal performance for each application.
- PDHβ is a 359 amino acid protein with an apparent molecular mass of ~40 kD.
Tissue Distribution: Ubiquitously expressed
Cellular Distribution: Mitochondrion and nucleus
- Beta subunit of the pyruvate dehydrogenase complex that catalyzes the conversion of pyruvate to acetyl-CoA and CO2.
- PDHβ forms heterotetramer with PDHα1 subunits.
- Cell Type
- Astrocytes, Mature Neurons, Microglia, Neural Stem Cells, Neurons, Oligodendrocytes
- Biology Area
- Cardiovascular Biology, Mitochondrial Function, Neurodegeneration, Neuroscience, Synaptic Biology
- Molecular Family
- Mitochondrial Markers
- Antigen References
- Bhandary S, et al. 2015. Epilepsy Res. 116:40
- Prasad C, et al. 2011. Brain Dev. 33(10):856
- Gene ID
- 5162 View all products for this Gene ID 68263 View all products for this Gene ID 289950 View all products for this Gene ID
- View information about PDHbeta on UniProt.org
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