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Antikörper

DHCR7 Antibody

Produktgrößen
50 ul
£186,00
CSB-PA110763-50UL
100 ul
£334,00
CSB-PA110763-100UL
Über dieses Produkt
SKU:
CSB-PA110763
Zusätzliche Namen:
DHCR7 antibody; D7SR antibody; 7-dehydrocholesterol reductase antibody; 7-DHC reductase antibody; EC 1.3.1.21 antibody; Delta7-sterol reductase antibody; Sterol Delta(7)-reductase antibody; Sterol reductase SR-2 antibody
Anwendung:
ELISA, Immunohistochemistry
Buffer:
PBS
Klonalität:
Polyclonal
Weitere Details:
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.
Host:
Rabbit
Immunogen:
Fusion protein of Human DHCR7
Isotyp:
IgG
Physischer Zustand:
Liquid
Aufreinigung:
Affinity Purified
Reaktivitäten:
Human, Mouse
Versandbedingungen:
Blue Ice
Lagerbedingungen:
-20[o]C/-70[o]C Avoid freeze/thaw cycles.
Hersteller:
Cusabio
Typ:
Antibody: Polyclonal Antibody