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 Omim: 602858 Human SwissProt: Q9UBM7 Human SwissProt: O88455 Mouse Unigene: 503134 Human |
產(chǎn)品圖片 | |