DLDBP FISH Probe
The DLDBP FISH probe is designed to hybridize to the DLDBP gene and is primarily used for detecting amplifications and deletions associated with the gene. This probe is FISH confirmed on normal peripheral blood metaphase spreads and interphase nuclei. The probe can be labeled in one of five colors. Each probe is sold in a 20 test kit (approximately 20 slides - 22x22 mm area) and includes hybridization buffer. Please note that due to design optimizations, prices are subject to change.
** This product is for in vitro and research use only. This product is not intended for diagnostic use.
| SKU | Test Kits | Buffer | Dye Color | Order Now |
|---|---|---|---|---|
| PDHX-20-OR (Standard Design) | 20 (40 μL) | 200 μL |  |
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| PDHX-20-RE | 20 (40 μL) | 200 μL |  |
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| PDHX-20-GO | 20 (40 μL) | 200 μL |  |
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| PDHX-20-GR | 20 (40 μL) | 200 μL |  |
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| PDHX-20-AQ | 20 (40 μL) | 200 μL |  |
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| PDHX-20-DIG | 20 (40 μL) | 200 μL |  |
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| PDHX-20-BIO | 20 (40 μL) | 200 μL |  |
Gene Summary
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.[provided by RefSeq, Oct 2009]
Gene Details
Gene Symbol: PDHX
Gene Name: Pyruvate Dehydrogenase Complex Component X
Chromosome: CHR11: 34937676-35017675
Locus: 11p13
FISH Probe Protocols
| Protocol, Procedure, or Form Name | Last Modified | Download |
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Customer Publications
Product Details
Product: DLDBP FISH Probe
Test Kits: 20 (40 μL)
ISH Buffer: 200 μL
SKU: PDHX-20-OR
Material Safety Data Sheet: MSDS.pdf
Turnaround Time: 7-10 Business Days
Shipping Time: 1-2 Day Expedited Shipping