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DHCR7 Rabbit pAb (bs-5057R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價(jià)

產(chǎn)品編號(hào) bs-5057R
英文名稱 DHCR7 Rabbit pAb
中文名稱 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.  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號(hào)
交叉反應(yīng) Mouse (predicted: Human,Rat,Cow,Chicken,Dog,Horse)
產(chǎn)品應(yīng)用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 54 kDa
檢測分子量
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DHCR7: 351-450/475 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
Q9UBM7

Gene ID:
1717

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



產(chǎn)品圖片
Sample: Liver (Mouse) Lysate at 40 ug Primary: Anti- DHCR7 (bs-5057R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 54 kD Observed band size: 54 kD
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