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Mouse Anti-PDHA1 Recombinant Antibody (2B10) (CBMAB-P1278-YC)

Provided herein is a Mouse monoclonal antibody against Human Pyruvate Dehydrogenase E1 Alpha 1 Subunit. The antibody can be used for immunoassay techniques, such as WB, IF, FC.
See all PDHA1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
2B10
Antibody Isotype
IgG2b
Application
WB, IF, FC

Basic Information

Immunogen
Full length human recombinant protein of human PDHA1 (NP_000275) produced in HEK293T cell
Specificity
Human
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
PBS, pH 7.3, 1% BSA, 50% glycerol, 0.02% sodium azide
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Pyruvate Dehydrogenase E1 Alpha 1 Subunit
Introduction
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.
Entrez Gene ID
5160
UniProt ID
P08559
Alternative Names
Pyruvate Dehydrogenase E1 Alpha 1 Subunit; Pyruvate Dehydrogenase E1 Component Subunit Alpha, Somatic Form, Mitochondrial; Pyruvate Dehydrogenase (Lipoamide) Alpha 1; Pyruvate Dehydrogenase Alpha 1; PDHE1-A Type I; EC 1.2.4.1; PHE1A;
Function
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links the glycolytic pathway to the tricarboxylic cycle.
Biological Process
Acetyl-CoA biosynthetic process from pyruvateManual Assertion Based On ExperimentIDA:UniProtKB
Glucose metabolic processIEA:UniProtKB-KW
Mitochondrial acetyl-CoA biosynthetic process from pyruvateIEA:Ensembl
Tricarboxylic acid cycleIEA:UniProtKB-KW
Cellular Location
Mitochondrion matrix
Involvement in disease
Pyruvate dehydrogenase E1-alpha deficiency (PDHAD):
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.
PTM
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.
Acetylation alters the phosphorylation pattern. Deacetylated by SIRT3 (By similarity).
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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