重组人ATP8B1蛋白(ab152604)

概述

  • 产品名称
    重组人ATP8B1蛋白
  • 蛋白长度
    Protein fragment

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • 种属
      Human
    • 序列
      INGQIYGDHRDASQHNHNKIEQVDFSWNTYADGKLAFYDHYLIEQIQSGK EPEVRQFFFLLAVCHTVMVDRTDGQLNYQAA
    • 氨基酸
      471 to 551
    • 标签
      proprietary tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab152604 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    Western blot

    ELISA

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • AT8B1_HUMAN
    • ATP8B1
    • ATPase class I type 8B member 1
    • ATPase, aminophospholipid transporter, class I, type 8B, member 1
    • ATPIC
    • BRIC
    • E1-E2 ATPase
    • Familial intrahepatic cholestasis type 1
    • FIC1
    • OTTHUMP00000163615
    • PFIC
    • PFIC1
    • Phospholipid transporting ATPase IC
    • Probable phospholipid-transporting ATPase IC
    see all
  • 功能
    May play a role in the transport of aminophospholipids from the outer to the inner leaflet of various membranes and the maintenance of asymmetric distribution of phospholipids in the canicular membrane. May have a role in transport of bile acids into the canaliculus, uptake of bile acids from intestinal contents into intestinal mucosa or both.
  • 组织特异性
    Found in most tissues except brain and skeletal muscle. Most abundant in pancreas and small intestine.
  • 疾病相关
    Defects in ATP8B1 are the cause of progressive familial intrahepatic cholestasis type 1 (PFIC1) [MIM:211600]; also known as Byler disease. PFIC1 is an autosomal recessive disorder, characterized by early infancy cholestasis, that may be initially episodic but progresses to malnutrition, growth retardation and end-stage liver disease before adulthood.
    Defects in ATP8B1 are the cause of benign recurrent intrahepatic cholestasis type 1 (BRIC1) [MIM:243300]; also known as Summerskill syndrome. 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. Patients are asymptomatic between episodes, both clinically and biochemically.
    Defects in ATP8B1 can be associated with intrahepatic cholestasis of pregnancy (ICP) [MIM:147480]; also known as pregnancy-related cholestasis. ICP is a multifactorial liver disorder of pregnancy. It presents during the second or, more commonly, the third trimestre of pregnancy with intense pruritus which becomes more severe with advancing gestation and cholestasis. Cholestasis results from abnormal biliary transport from the liver into the small intestine. ICP causes fetal distress, spontaneous premature delivery and intrauterine death. ICP patients have spontaneous and progressive disappearance of cholestasis after delivery.
  • 序列相似性
    Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IV subfamily.
  • 细胞定位
    Membrane.
  • Information by UniProt

图片

  • ab152604 on a 12.5% SDS-PAGE stained with Coomassie Blue.

文献

ab152604 has not yet been referenced specifically in any publications.

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