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: ICC/IF, WB, IHC-Pmore details
Species reactivityReacts with: Mouse
Predicted to work with: Rat, Cow, Human, Pig, Chimpanzee, Cynomolgus monkey, Orangutan
Recombinant full length protein corresponding to Human Pyruvate Dehydrogenase E1-alpha subunit aa 30-390.
FANDATFEIKKCDLHRLEEGPPVTTVLTREDGLKYYRMMQTVRRMELKAD QLYKQKIIRGFCHLCDGQEACCVGLEAGINPTDHLITAYRAHGFTFTRGL SVREILAELTGRKGGCAKGKGGSMHMYAKNFYGGNGIVGAQVPLGAGIAL ACKYNGKDEVCLTLYGDGAANQGQIFEAYN MAALWKLPCIFICENNRY GMGTSVERAAASTDYYKRGDFIPGLRVDGMDILCVREATRFAAAYCRSGK GPILMELQTYRYHGHSMSDPGVSYRTREEIQEVRSKSDPIMLLKDRMVNS NLASVEELKEIDVEVRKEIEDAAQFATADPEPPLEELGYHIYSSDPPFEV RGANQWIKFKSV S
Database link: P08559
- Mouse heart and Mouse skeletal muscle cell extracts.
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: 7.30
Preservative: 0.02% Sodium azide
Constituents: 50% Glycerol, 49% PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab180759 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use at an assay dependent concentration.|
|WB||1/500 - 1/2000. Predicted molecular weight: 43 kDa.|
|IHC-P||1/50 - 1/200.
ab171870 - Rabbit polyclonal IgG, 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
Immunocytochemistry/Immunofluorescence analysis of U2OS cells using ab180759. Blue DAPI for nuclear staining.
All lanes : Anti-Pyruvate Dehydrogenase E1-alpha subunit antibody (ab180759) at 1/500 dilution
Lane 1 : Mouse heart cell extract
Lane 2 : Mouse skeletal muscle cell extract
Predicted band size: 43 kDa
This product has been referenced in:
- Wang Z et al. Curcumin restrains hepatic glucose production by blocking cAMP/PKA signaling and reducing acetyl CoA accumulation in high-fat diet (HFD)-fed mice. Mol Cell Endocrinol 474:127-136 (2018). WB ; Mouse . Read more (PubMed: 29499209) »
- Kennedy BE et al. Presymptomatic Alterations in Amino Acid Metabolism and DNA Methylation in the Cerebellum of a Murine Model of Niemann-Pick Type C Disease. Am J Pathol : (2016). Read more (PubMed: 27083515) »