Key features and details
- HRP Mouse monoclonal [9H9AF5] to PDHA1
- Suitable for: WB
- Reacts with: Human
- Conjugation: HRP
- Isotype: IgG1
Product nameHRP Anti-PDHA1 antibody [9H9AF5]
See all PDHA1 primary antibodies
DescriptionHRP Mouse monoclonal [9H9AF5] to PDHA1
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Cow
Recombinant full length protein corresponding to Human PDHA1.
- WB: Human heart mitochondrial lysate and HepG2 whole cell lysate.
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Storage instructionsShipped at 4°C. Upon delivery aliquot. Store at +4°C. Avoid freeze / thaw cycle. Store In the Dark.
Storage bufferpH: 7.40
Preservative: 0.1% 10% Proclin 300 Solution
Constituents: 30% Glycerol, 1% BSA, PBS
Concentration information loading...
Purification notesab110330 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
Light chain typekappa
Our Abpromise guarantee covers the use of ab197956 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/3000. Detects a band of approximately 43 kDa (predicted molecular weight: 43 kDa).|
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 : HRP Anti-PDHA1 antibody [9H9AF5] (ab197956) at 1/3000 dilution
Lane 1 : Human heart tissue lysate - mitochondrial extract (ab110337) at 10 µg
Lane 2 : HepG2 (Human hepatocellular liver carcinoma cell line) Whole Cell Lysate at 20 µg
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 43 kDa
Observed band size: 43 kDa
Exposure time: 8 minutes
This blot was produced using a 4-12% Bis-tris gel under the MOPS buffer system. The gel was run at 200V for 50 minutes before being transferred onto a Nitrocellulose membrane at 30V for 70 minutes. The membrane was then blocked for an hour using 3% milk before being incubated with ab197956 overnight at 4°C. Antibody binding was visualised using ECL development solution ab133406.
ab197956 has not yet been referenced specifically in any publications.