Rabbit Anti-PDHA1 antibody | Sunlong Medical

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Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial; ODPA_HUMAN; EC:1.2.4.1; PDHE1-A type I; pyruvate dehydrogenase E1 subunit alpha 1; PDHA; PDHAD; PHE1A; E1alpha; PDHCE1A; PDH-E1α;

Cat:

SL4034R

Species Reactivity：

Human,Mouse,Rat,(predicted: Chicken,Dog,Pig,Cow,Horse,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human PDHA1:251-350/388

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

WB=1:500-2000ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Product Overview：

Sample: Kidney (Mouse) Lysate at 40 ugPrimary: Anti-PDHA1 (SL4034R) at 1/300 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 43 kDObserved band size: 43 kDSample: Heart (Mouse) Lysate at 40 ugPrimary: Anti-PDHA1 (SL4034R) at 1/300 dilutionSecondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilutionPredicted band size: 43 kDObserved band size: 43 kDParaformaldehyde-fixed, paraffin embedded (Rat heart); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PDHA1) Polyclonal Antibody, Unconjugated (SL4034R) at 1:400 overnight at 4°C, followed by a conjugated secondary antibody (sp-0023) for 20 minutes and DAB staining.

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2010]

Function:
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.

Subunit:
Tetramer of 2 alpha and 2 beta subunits.

Subcellular Location:
Mitochondrion matrix.

Tissue Specificity:
Testis. Expressed in postmeiotic spermatogenic cells.

Post-translational modifications:
Phosphorylation at Ser-232, Ser-293 and Ser-300 by PDK family kinases inactivates the enzyme; for this phosphorylation at a single site is sufficient. Dephosphorylation at all three sites, i.e. at Ser-232, Ser-293 and Ser-300, is required for reactivation.

DISEASE:
Pyruvate dehydrogenase E1-alpha deficiency (PDHAD) [MIM:312170]: An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis. Note=The disease is caused by mutations affecting the gene represented in this entry.
X-linked Leigh syndrome (X-LS) [MIM:308930]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.

SWISS:
P08559

Gene ID:
5160

Database links:

Entrez Gene: 407109 Cow

Entrez Gene: 5160 Human

Entrez Gene: 18597 Mouse

Entrez Gene: 29554 Rat

SwissProt: A7MB35 Cow

SwissProt: P08559 Human

SwissProt: P35486 Mouse

SwissProt: P26284 Rat

Picture

Sample: Heart (Mouse) Lysate at 40 ug
Primary: Anti-PDHA1 (SL4034R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 43 kD
Observed band size: 43 kD

Paraformaldehyde-fixed, paraffin embedded (Rat heart); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PDHA1) Polyclonal Antibody, Unconjugated (SL4034R) at 1:400 overnight at 4°C, followed by a conjugated secondary antibody (sp-0023) for 20 minutes and DAB staining.

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