| 產(chǎn)品編號 | bs-12440R |
| 英文名稱 | ABCB11 Rabbit pAb |
| 中文名稱 | 膽汁酸鹽輸出泵抗體 |
| 別 名 | ABCBB_HUMAN; Bile salt export pump; BSEP; EC:7.6.2.-; ATP-binding cassette sub-family B member 11; PGY4; SPGP; ABC16; BRIC2; PFIC2; PFIC-2; |
| 研究領(lǐng)域 | 腫瘤 細胞生物 信號轉(zhuǎn)導(dǎo) 轉(zhuǎn)運蛋白 新陳代謝 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Rabbit) |
| 產(chǎn)品應(yīng)用 | ICC/IF=1:100-500
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 146 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ABCB11: 1001-1100/1321 <Cytoplasmic> |
| 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 | 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 membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is the major canalicular bile salt export pump in man. Mutations in this gene cause a form of progressive familial intrahepatic cholestases which are a group of inherited disorders with severe cholestatic liver disease from early infancy. [provided by RefSeq, Jul 2008] Function: Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes. Subunit: Interacts with HAX1. Subcellular Location: Membrane; Multi-pass membrane protein. Tissue Specificity: Expressed predominantly, if not exclusively in the liver, where it was further localized to the canalicular microvilli and to subcanalicular vesicles of the hepatocytes by in situ. DISEASE: Defects in ABCB11 are the cause of progressive familial intrahepatic cholestasis type 2 (PFIC2) [MIM:601847]. PFIC2 is an inherited liver disease of childhood which is characterized by cholestasis and normal serum gamma-glutamyltransferase activity. Defects in ABCB11 are also found in cases of chronic intrahepatic cholestasis without obvious familial history of chronic liver disease. Defects in ABCB11 are the cause of benign recurrent intrahepatic cholestasis type 2 (BRIC2) [MIM:605479]. BRIC is characterized by intermittent episodes of cholestasis without progression to liver failure. There is initial elevation of serum bile acids, followed by cholestatic jaundice which generally spontaneously resolves after periods of weeks to months. The cholestatic attacks vary in severity and duration and patients are asymptomatic between episodes, both clinically and biochemically. Similarity: Belongs to the ABC transporter superfamily. ABCB family. Multidrug resistance exporter (TC 3.A.1.201) subfamily. Contains 2 ABC transmembrane type-1 domains. Contains 2 ABC transporter domains. SWISS: O95342 Gene ID: 8647 Database links: Entrez Gene: 8647 Human Entrez Gene: 27413 Mouse SwissProt: O95342 Human SwissProt: Q9QY30 Mouse |
