Rabbit polyclonal PDHX antibody (A6426)

$69.00$259.00

Reactivity: Human
Applications: WB,IHC,IF
Conjugation: Unconjugated
Supplier: ABclonal Inc.

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Rabbit polyclonal PDHX antibody (A6426)

Supplier: ABclonal Inc.

Recommended applications: WB,IHC,IF

Recommended dilution:

WB 1:500 – 1:2000 IHC 1:50 – 1:200 IF 1:10 – 1:100

Recommended protocols: check protocols

Image descriptions:

Click or hover above images to see image description for Rabbit polyclonal PDHX antibody.

Alternative names:

Check alternative names for the antibody

Expand

E3BP antibody, OPDX antibody, PDX1 antibody, proX antibody, DLDBP antibody
70 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody|AI481367 antibody|BOS_15094 antibody|Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex antibody|Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex antibody|DLAT antibody|DLDBP antibody|DLTA antibody|E3BP antibody|M2 antigen complex 70 kDa subunit antibody|MGC143185 antibody|mitochondrial antibody|ODP2_HUMAN antibody|OPDX antibody|PBC antibody|PDC-E2 antibody|PDCE2 antibody|PDX1 antibody|proX antibody|Pyruvate dehydrogenase complex component E2 antibody|Pyruvate dehydrogenase complex, component X antibody|RP23-203N8.2 antibody|Anti-Pyruvate dehydrogenase E2/E3bp antibody [13G2AE2BH5] (ab110333)

SCBT cat No: sc-365276|sc-271534|sc-166899|sc-16892|sc-271352|sc-32925|sc-16890|

Name

Rabbit polyclonal PDHX antibody

Catalogue No.

A6426

Reactivity

Human

Immunogen

Recombinant protein of human PDHX

Host

Rabbit

Applications

WB, IHC, IF

Recommended dilution

WB 1:500 – 1:2000
IHC 1:50 – 1:200
IF 1:10 – 1:100

Clonality

Polyclonal

Conjugation

Unconjugated

Isotype

IgG

Molecular weight

Predicted: 54kDa/Observed: Refer to Figures

Formulation

PDHX antibody was tube-contained.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Purification

PDHX antibody was purified using affinity purification.

Storage

Store at -20 Celsius degree. Avoid freeze / thaw cycles.

Alternative antibody names

E3BP antibody, OPDX antibody, PDX1 antibody, proX antibody, DLDBP antibody

Database links

Human UniProt/Swiss-Prot:O00330

Protein names

E3BP, OPDX, PDX1, proX, DLDBP

Protein function

Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.

Protein sequence and domain

Belongs to the 2-oxoacid dehydrogenase family.; Contains 1 lipoyl-binding domain.

Protein post-translational modifications

Delipoylated at Lys-97 by SIRT4, delipoylation decreases the PHD complex activity.

Protein cellular localization

Mitochondrion matrix.

Background

The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

Note

AntibodyPlus can customize PDHX antibody according to your requirement, including bulk product size,etc. Please contact info@antibodyplus.com. AntibodyPlus provides antibody trial sample for your own antibody validation.

Product type

Primary antibody

Supplier

ABclonal Inc.

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