| Product Name | Human 7-dehydrocholesterol reductase (DHCR7) ELISA Kit |
|---|---|
| Description | Human 7-dehydrocholesterol reductase (DHCR7) ELISA Kit has high sensitivity and excellent specificity for detection of Human DHCR7. No significant cross-reactivity or interference between Human DHCR7 and analogues was observed. 7-dehydrocholesterol reductasean enzyme that removes the C(7-8) double bond in the B ring of sterols and catalyzes the conversion of 7-dehydrocholesterol to cholesterol. This gene is ubiquitously expressed and its transmembrane protein localizes to the endoplasmic reticulum membrane and nuclear outer membrane. Mutations in this gene cause Smith-Lemli-Opitz syndrome (SLOS); a syndrome that is metabolically characterized by reduced serum cholesterol levels and elevated serum 7-dehydrocholesterol levels and phenotypically characterized by mental retardation, facial dysmorphism, syndactyly of second and third toes, and holoprosencephaly in severe cases to minimal physical abnormalities and near-normal intelligence in mild cases. Alternative splicing results in multiple transcript variants that encode the same protein. This Human 7-dehydrocholesterol reductase (DHCR7) ELISA Kit employs a two-site sandwich ELISA to quantitate DHCR7 in samples. An antibody specific for DHCR7 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyDHCR7 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for DHCR7 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of DHCR7 bound in the initial step. The color development is stopped and the intensity of the color is measured. |
| Synonyms | DHCR7, SLOS, delta-7-dehydrocholesterol reductase, sterol delta-7-reductase |
| Method | Sandwich ELISA |
| Detection Range | Please inquire |
| Sensitivity | Please inquire |
| Reactivity | Human |
| Sample Types | Cell culture supernatants, Serum, Plasma, Other biological fluids |
| Gene Id | 1717 |
| Storage | 2-8°C |
| Background | 7-dehydrocholesterol reductasean enzyme that removes the C(7-8) double bond in the B ring of sterols and catalyzes the conversion of 7-dehydrocholesterol to cholesterol. This gene is ubiquitously expressed and its transmembrane protein localizes to the endoplasmic reticulum membrane and nuclear outer membrane. Mutations in this gene cause Smith-Lemli-Opitz syndrome (SLOS); a syndrome that is metabolically characterized by reduced serum cholesterol levels and elevated serum 7-dehydrocholesterol levels and phenotypically characterized by mental retardation, facial dysmorphism, syndactyly of second and third toes, and holoprosencephaly in severe cases to minimal physical abnormalities and near-normal intelligence in mild cases. Alternative splicing results in multiple transcript variants that encode the same protein. |
| Supplier | Abbkine |
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