Key features and details
- Mouse monoclonal [8D10E6] to PDHA1
- Suitable for: WB, ICC/IF, Flow Cyt
- Knockout validated
- Reacts with: Mouse, Rat, Cow, Human, Caenorhabditis elegans, Drosophila melanogaster
- Isotype: IgG1
Product nameAnti-PDHA1 antibody [8D10E6]
See all PDHA1 primary antibodies
DescriptionMouse monoclonal [8D10E6] to PDHA1
Tested applicationsSuitable for: WB, ICC/IF, Flow Cytmore details
Species reactivityReacts with: Mouse, Rat, Cow, Human, Caenorhabditis elegans, Drosophila melanogaster
Predicted to work with: Zebrafish
Full length native protein (purified). This information is considered to be commercially sensitive.
- WB: Isolated mitochondria from human, bovine, rat and mouse heart. HepG2 (human liver hepatocellular carcinoma cell line) cell lysate. FACS: HeLa (human epithelial cell line from cervix adenocarcinoma) and HL-60 (human promyelocytic leukemia cell line) cells.
This antibody clone is manufactured by Abcam.
Product was previously marketed under the MitoSciences sub-brand.
Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.
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. Do Not Freeze.
Storage bufferPreservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
Purification notesab110334 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
Light chain typekappa
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Energy Metabolism
Our Abpromise guarantee covers the use of ab110334 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.5 - 1 µg/ml. Predicted molecular weight: 43 kDa.|
|ICC/IF||Use a concentration of 1 µg/ml. (heat-induced antigen-retrieval improves signal)|
|Flow Cyt||Use a concentration of 1 µg/ml.
ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
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
- ODPA_HUMAN antibody
- PDH antibody
- PDHA antibody
All lanes : Anti-PDHA1 antibody [8D10E6] (ab110334) at 0.5 µg/ml
Lane 1 : Wild-type HeLa whole cell lysate
Lane 2 : PDHA1 knockout HeLa whole cell lysate
Lane 3 : HEK-293 whole cell lysate
Lysates/proteins at 20 µg per lane.
Predicted band size: 43 kDa
Observed band size: 43 kDa
Lanes 1 - 3: Merged signal (red and green). Green - ab110334 observed at 43 kDa. Red - loading control, ab52866, observed at 50 kDa.
ab110334 was shown to recognize PDHA1 in wild-type HeLa cells as signal was lost at the expected MW in PDHA1 knockout cells. Additional cross-reactive bands were observed in the wild-type and knockout cells. Wild-type and PDHA1 knockout samples were subjected to SDS-PAGE. Ab110334 and ab52866 (Rabbit anti alpha Tubulin loading control) were incubated overnight at 4°C at 0.5 ug/ml and 1/20000 dilution respectively. Blots were developed with Goat anti-Mouse IgG H&L (IRDye® 800CW) preabsorbed ab216772 and Goat anti-Rabbit IgG H&L (IRDye® 680RD) preabsorbed ab216777 secondary antibodies at 1/20000 dilution for 1 hour at room temperature before imaging.
All lanes : Anti-PDHA1 antibody [8D10E6] (ab110334) at 1 µg/ml
Lane 1 : Isolated mitochondria from human heart at 5 µg
Lane 2 : Isolated mitochondria from bovine heart at 1 µg
Lane 3 : Isolated mitochondria from rat heart at 10 µg
Lane 4 : Isolated mitochondria from mouse heart at 10 µg
Lane 5 : HepG2 cell lysate at 20 µg
Predicted band size: 43 kDa
Immunocytochemistry analysis using ab110334 at 1 µg/ml staining PDHA1 in HeLa (human epithelial cell line from cervix adenocarcinoma) cells (4% paraformaldehyde fixed and Triton X-100 permeabilized).
The secondary antibody was (green) Alexa Fluor® 488 goat anti-mouse IgG (H+L) used at a 1/1000 dilution. DAPI was used to stain the cell nuclei (blue).
Flow cytometric analysis using ab110334 at 1 µg/ml staining PDHA1 in HL-60 (human promyelocytic leukemia cell line) cells (blue). Isotype control antibody (red).
ab110334 has been referenced in 47 publications.
- Hutto RA et al. Increasing Ca2+ in photoreceptor mitochondria alters metabolites, accelerates photoresponse recovery, and reveals adaptations to mitochondrial stress. Cell Death Differ 27:1067-1085 (2020). PubMed: 31371786
- Wang X et al. Conditional knockout of pyruvate dehydrogenase in mouse pancreatic ß-cells causes morphological and functional changes. Mol Med Rep 21:1717-1726 (2020). PubMed: 32319629
- Kozlov AM et al. Lactate preconditioning promotes a HIF-1a-mediated metabolic shift from OXPHOS to glycolysis in normal human diploid fibroblasts. Sci Rep 10:8388 (2020). PubMed: 32433492
- Jiang YF et al. Coordinated organization of mitochondrial lamellar cristae and gain of COX function during mitochondrial maturation in Drosophila. Mol Biol Cell 31:18-26 (2020). PubMed: 31746672
- Purroy R et al. Frataxin-deficient cardiomyocytes present an altered thiol-redox state which targets actin and pyruvate dehydrogenase. Redox Biol 32:101520 (2020). PubMed: 32279039