Key features and details
- Rabbit polyclonal to Pyruvate Dehydrogenase E1-alpha subunit
- Suitable for: WB, IHC-P, IHC - Wholemount
- Reacts with: Zebrafish
- Isotype: IgG
Product nameAnti-Pyruvate Dehydrogenase E1-alpha subunit antibody
See all Pyruvate Dehydrogenase E1-alpha subunit primary antibodies
DescriptionRabbit polyclonal to Pyruvate Dehydrogenase E1-alpha subunit
Tested applicationsSuitable for: WB, IHC-P, IHC - Wholemountmore details
Species reactivityReacts with: Zebrafish
Recombinant fragment within Zebrafish Pyruvate Dehydrogenase E1-alpha subunit aa 62-348. The exact sequence is proprietary.
Database link: Q6P948
- Zebrafish embryo; zebrafish eye whole cell lysate; zebrafish kidney tissue.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.00
Preservative: 0.025% Proclin 300
Constituents: PBS, 20% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab210533 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/3000. Predicted molecular weight: 44 kDa.|
|IHC-P||1/100 - 1/1000.
Suggested antigen retrieval using 1mM EDTA-NaOH (pH8.0) 95ºC,1Hr
|IHC - Wholemount||1/100 - 1/500.|
FunctionThe pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3).
Involvement in diseaseDefects in PDHA1 are a cause of pyruvate decarboxylase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (X-LS).
Defects in PDHA1 are the cause of X-linked Leigh syndrome (X-LS) [MIM:308930]. 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. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes.
Cellular localizationMitochondrion matrix.
- Information by UniProt
- Entrez Gene: 406702 Zebrafish
- ODPA_HUMAN antibody
- PDH antibody
- PDHA antibody
Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab210533) at 1/1000 dilution + zebrafish eye whole cell lysate at 30 µg
Predicted band size: 44 kDa
Immunohistochemical analysis of paraffin-embedded Zebrafish kidney tissue labeling Pyruvate Dehydrogenase E1-alpha with ab210533 at 1/300 dilution.
Immunohistochemical analysis (wholemount) of zebrafish embryo labeling Pyruvate Dehydrogenase E1-alpha with ab210533 at 1/200 dilution.
ab210533 has not yet been referenced specifically in any publications.