Catalog_no | AB0924 |
Product_name | DHCR7 Antibody (C-term) |
Category | 抗原抗体 |
Applications | WB, IHC-P, FC |
Reactivity | H, M |
Size | 100μL/50μL |
Price | 2000.00/1100.00 |
immunogen | HUMAN:437-463 |
Specificity | This DHCR7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 437-463 amino acids from the C-terminal region of human DHCR7. |
Dilution | IF,1:25;WB,1:2000;WB,1:1000;IHC-P,1:50~100; |
Purification | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
othername | 7-dehydrocholesterol reductase, 7-DHC reductase, Putative sterol reductase SR-2, Sterol Delta(7)-reductase, DHCR7, D7SR |
Isotype | Rabbit Ig |
Background | 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. |
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