别名:SLOS应用:IHC
反应种属:Human, Mouse
规格:50μl/100μl
| Description |
|---|
| This gene encodes an 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. |
| Specification | |
|---|---|
| Aliases | SLOS |
| Swissprot | Q9UBM7 |
| Host/Isotype | Rabbit IgG |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse |
| Immunogen | Fusion protein of human DHCR7 |
| Formulation | pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
| Application | |
|---|---|
| IHC | 1/25-1/100 |
| ELISA | 1/1000-1/2000 |
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The image is immunohistochemistry of paraffin-embedded Human colon cancer tissue using P08238(DHCR7 Antibody) at dilution 1/20. (Original magnification: ×200) |
本公司的所有产品仅用于科学研究或者工业应用等非医疗目的,不可用于人类或动物的临床诊断或治疗,非药用,非食用。
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本公司的所有产品仅用于科学研究或者工业应用等非医疗目的,不可用于人类或动物的临床诊断或治疗,非药用,非食用。
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