PDHX Rabbit Polyclonal Antibody (CAB6426)
- SKU:
- CAB6426
- Product Type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
Product Name: | PDHX Rabbit Polyclonal Antibody |
SKU: | CAB6426 |
Size: | 20uL, 100uL |
Isotype: | IgG |
Host Species: | Rabbit |
Reactivity: | Human,Mouse,Rat |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2). |
Sequence: | MAAS WRLG CDPR LLRY LVGF PGRR SVGL VKGA LGWS VSRG ANWR WFHS TQWL RGDP IKIL MPSL SPTM EEGN IVKW LKKE GEAV SAGD ALCE IETD KAVV TLDA SDDG ILAK IVVE EGSK NIRL GSLI GLIV EEGE DWKH VEIP KDVG PPPP VSKP SEPR PSPE PQIS IPVK KEHI PGTL RFRL SPAA RNIL EKHS LDAS QGTA TGPR GIFT KEDA LKLV QLKQ TGKI TESR PTPA PTAT PTAP SPLQ ATAG PSYP RPVI PPVS TPGQ PNAV GTFT EIPA SNIR RVIA KRLT ESKS TVPH |
Tested Applications: | WB IP ELISA |
Recommended Dilution: | WB,1:500 - 1:2000 IP,0.5μg-4μg antibody for 400μg-600μg extracts of whole cells |
Synonyms: | E3BP; OPDX; PDX1; proX; DLDBP; PDHXD; PDHX |
Positive Sample: | SW620,HepG2,U-251MG,MCF7,Mouse heart,Mouse testis,Mouse brain,Rat kidney,Rat heart |
Conjugate: | Unconjugated |
Cellular Localization: | Mitochondrion matrix. |
Calculated MW: | 54kDa |
Observed MW: | 54kDa |
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.
Purification Method: | Affinity purification |
Gene ID: | 8050 |
Storage Buffer: | Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3. |