PDHX Antibody
| Code | Size | Price |
|---|
| PSI-22-212-50uL | 50uL | £433.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Isotype: IgG
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Shipping:
Blue Ice or RT
Storage:
Store at -20°C. Avoid freeze / thaw cycles.
Documents
Further Information
Additional Names:
PDHX, E3BP, OPDX, PDX1, proX, DLDBP, pyruvate dehydrogenase complex, component X, E3-binding protein, lipoyl-containing component X
Application Note:
WB: 1:500 - 1:2000
IF: 1:10 - 1:100
IF: 1:10 - 1:100
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.
Buffer:
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Concentration:
batch dependent
Conjugate:
Unconjugated
DISCLAIMER:
Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.
Immunogen:
Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2).
NCBI Gene ID #:
8050
NCBI Official Name:
pyruvate dehydrogenase complex, component X
NCBI Official Symbol:
PDHX
NCBI Organism:
Homo sapiens
Physical State:
Liquid
PREDICTED MOLECULAR WEIGHT:
Observed: 54kDa
Purification:
Affinity purification
Research Area:
Cancer, Signal Transduction
Swissprot #:
O00330
User NOte:
Optimal dilutions for each application to be determined by the researcher.