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Rabbit Anti-DHCR7/PE-Cy5 Conjugated antibody (bs-5057R-PE-Cy5)
訂購熱線:400-901-9800
訂購郵箱:sales@www.ssxsbs.com
訂購QQ:  400-901-9800
技術支持:techsupport@www.ssxsbs.com
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-5057R-PE-Cy5
英文名稱 Rabbit Anti-DHCR7/PE-Cy5 Conjugated antibody
中文名稱 PE-Cy5標記的脫氫膽固醇還原酶7抗體
別    名 7 dehydrocholesterol reductase; 7 DHC reductase; Delta 7 dehydrocholesterol reductase; Putative sterol reductase SR 2; Sterol Delta; DHCR7_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,  (predicted: Human, Rat, Chicken, Dog, Cow, Horse, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 54kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DHCR7
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
The DHCR7 gene encodes delta-7-sterol reductase (EC 1.3.1.21), the penultimate enzyme of mammalian sterol biosynthesis that converts 7-dehydrocholesterol (7-DHC) to cholesterol.

Function:
Production of cholesterol by reduction of C7-C8 double bond of 7-dehydrocholesterol (7-DHC).

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

Tissue Specificity:
Most abundant in adrenal gland, liver, testis, and brain.

DISEASE:
Defects in DHCR7 are the cause of Smith-Lemli-Opitz syndrome (SLOS) [MIM:270400]; also known as SLO syndrome or RSH syndrome. SLOS is an autosomal recessive frequent inborn disorder of sterol metabolism with characteristic congenital malformations and dysmorphias. All patients suffer from mental retardation. Children with SLOS have elevated serum 7-dehydrocholesterol (7-DHC) levels and low serum cholesterol levels. SLOS occurs in relatively high frequency: approximately 1 in 20,000 to 30,000 births in populations of northern and central European background. Historically, a clinical distinction often was made between classic ('type I') SLOS and the more severely affected ('type II') patients. There is, in reality, a clinical and biochemical continuum from mild to severe SLOS.

Similarity:
Belongs to the ERG4/ERG24 family.

Database links:

Entrez Gene: 1717 Human

Entrez Gene: 13360 Mouse

Entrez Gene: 64191 Rat

Omim: 602858 Human

SwissProt: Q9UBM7 Human

SwissProt: O88455 Mouse

SwissProt: Q9Z2Z8 Rat

Unigene: 503134 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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