Human Pyruvate Dehydrogenase Complex Component X (PDHX) ELISA Kit
Por favor contáctenos para obtener información detallada sobre el precio y disponibilidad.
Description
Human Pyruvate Dehydrogenase Complex Component X (PDHX) ELISA Kit is an ELISA Kit for the in vitro quantitative measurement of Human Pyruvate Dehydrogenase Complex Component X (PDHX) concentrations in tissue homogenates, cell lysates and other biological fluids.
Documents del producto
Product specifications
| Category | ELISA Kits |
| Immunogen Target | Pyruvate Dehydrogenase Complex Component X (PDHX) |
| Reactivity | Human |
| Detection Method | Colorimetric |
| Assay Data | Quantitative |
| Assay Type | Sandwich |
| Test Range | 1.56 ng/ml - 100 ng/ml |
| Sensitivity | < 0.54 ng/ml |
| Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
| Size 1 | 96 tests |
| Size 2 | 5 × 96 tests |
| Size 3 | 10 × 96 tests |
| Form | Lyophilized |
| Tested Applications | ELISA |
| Sample Type | Tissue homogenates, cell lysates and other biological fluids. |
| Availability | Shipped within 5-12 working days. The validity for this kit is at least 6 months. Up to 12 months validity can be provided on request. |
| Storage | Shipped at 4 °C. Upon receipt, store the kit according to the storage instruction in the kit's manual. |
| Dry Ice | No |
| UniProt ID | O00330 |
| Gene ID | 8050 |
| OMIM | 245349 |
| Alias | DLDBP, E3BP, OPDX, PDX1, proX, pyruvate dehydrogenase complex component X, PDHXD |
| Background | Elisa kits for PDHX |
| Status | RUO |
| Note | Validity: The validity for this kit is at least 6 months. Up to 12 months validity can be provided on request. This product is for research use only. The range and sensitivity is subject to change. Please contact us for the latest product information. For accurate results, sample concentrations must be diluted to mid-range of the kit. If you require a specific range, please contact us in advance or write your request in your order comments. Please note that our ELISA and CLIA kits are optimised for detection of native samples, rather than recombinant proteins. We are unable to guarantee detection of recombinant proteins, as they may have different sequences or tertiary structures to the native protein. |
Descripción
PDHX is a component of the pyruvate dehydrogenase complex (PDC) located in the mitochondrial matrix, where it plays a critical role in linking glycolysis to the tricarboxylic acid (TCA) cycle by catalyzing the conversion of pyruvate to acetyl-CoA. PDHX functions as the E3-binding protein, anchoring dihydrolipoamide dehydrogenase (E3) to the PDC core and facilitating the transfer of reducing equivalents between PDC subunits. It is essential for the stability and activity of the PDC, ensuring efficient energy production and metabolic homeostasis. Mutations in PDHX are associated with pyruvate dehydrogenase deficiency, a condition characterized by lactic acidosis, developmental delay, and neurological dysfunction due to impaired mitochondrial energy metabolism. PDHX is expressed ubiquitously in energy-demanding tissues such as the brain, muscle, and liver, where it supports ATP production and metabolic adaptation. Knockout studies demonstrate reduced PDC activity, metabolic imbalances, and severe defects in energy-dependent processes, underscoring its importance in cellular respiration and energy metabolism.
Related Products
anti- PDHX antibody
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.
Ver Producto
Recombinant Human PDHX
Ver Producto
Pyruvate Dehydrogenase Complex Component X (PDHX) Antibody
PDHX Antibody is a Rabbit Polyclonal antibody against PDHX. 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.
Ver Producto