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Rabbit Anti-BSEP/BF350 Conjugated antibody (bs-1954R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-1954R-BF350
英文名稱 Rabbit Anti-BSEP/BF350 Conjugated antibody
中文名稱 BF350標記的膽汁酸鹽輸出泵/ATP結合盒轉運蛋白11抗體
別    名 ABC member 16, MDR/TAP subfamily; ABCB1; Abcb11; ABCBB_HUMAN; ATP binding cassette sub family B (MDR/TAP) member 11; ATP binding cassette sub family B member 11; ATP-binding cassette sub-family B member 11; Bile salt export pump; BRIC2; Bsep; PGY4; progressive familial intrahepatic cholestasis 2; Sister of P glycoprotein; Spgp.  
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  通道蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Rabbit, )
產品應用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 145kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BSEP
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:
ATP-binding cassette (ABC) transporters are an evolutionarily conserved family of proteins that catalyze the transport of molecules across extra- and intracellular membranes through the energy of ATP hydrolysis. ABC genes comprise seven subfamilies, designated ABC1, Mdr/TAP, MRP, ALD, OABP, GCN20 and White. The secretion of bile salt molecules from blood into bile is a major driving force for bile formation. Bile salt export pump (BSEP) is a member of the Mdr/TAP subfamily of ABC transporters that mediates the transport of bile acids across the hepatocyte canalicular membrane and regulates bile acid-dependent bile secretion. BSEP contains putative phosphorylation sites for protein kinase A, protein kinase C (PKC) and Ca2+-calmodulin dependent kinase II, whose regulation may be dependent on bile salt concentration.

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.

Database links:

Entrez Gene: 8647 Human

Entrez Gene: 27413 Mouse

Omim: 603201 Human

SwissProt: O95342 Human

SwissProt: Q9QY30 Mouse

Unigene: 658439 Human

Unigene: 439855 Mouse



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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