Key features and details
- Rabbit polyclonal to PDHA1
- Suitable for: WB, IHC-P, IHC - Wholemount
- Reacts with: Zebrafish
- Isotype: IgG
Product nameAnti-PDHA1 antibody
See all PDHA1 primary antibodies
DescriptionRabbit polyclonal to PDHA1
Tested applicationsSuitable for: WB, IHC-P, IHC - Wholemountmore details
Species reactivityReacts with: Zebrafish
Recombinant fragment within Zebrafish PDHA1 aa 62-348. The exact sequence is proprietary.
Database link: Q6P948
- Zebrafish embryo; zebrafish eye whole cell lysate; zebrafish kidney tissue.
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Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.
We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.
In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.
We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
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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: 79% PBS, 20% Glycerol (glycerin, glycerine)
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-PDHA1 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 PDHA1 with ab210533 at 1/300 dilution.
Immunohistochemical analysis (wholemount) of zebrafish embryo labeling PDHA1 with ab210533 at 1/200 dilution.
ab210533 has not yet been referenced specifically in any publications.