深圳欣博盛生物科技有限公司 a1

DHCR7 antibody
货号:GTX04343 规格:400μl 目录价:¥5600
产品详情
* 以下信息仅供参考,详情请以原厂网站为准
产品名称:
DHCR7 antibody
别名:
7-dehydrocholesterol reductase , SLOS
反应种属:
Human, Mouse
宿主来源:
Rabbit
实验应用:
ICC/IF, IHC-P, WB
同种型:
IgG
免疫原:
KLH conjugated synthetic peptide between 437-463 amino acids from the C-terminal region of human DHCR7.
克隆性:
Polyclonal
克隆号:
纯化方式:
Protein A purified followed by peptide affinity purification
偶联:
Unconjugated
产品浓度:
Batch dependent (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
预期分子量:
54
产品形式:
Liquid
存储溶液:
PBS, 0.09% (W/V) sodium azide.
生产商:
GeneTex
功能与背景:
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 cognitive disability, 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.[provided by RefSeq, Aug 2009]
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