ATP8B1, Polyclonal Antibody

Phospholipid-transporting ATPase IC; ATPase class I type 8B member 1; Familial intrahepatic cholestasis type 1; P4-ATPase flippase complex alpha subunit A; ATP8B1; ATPIC; FIC1; PFIC

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

>95%, Protein G purified

Liquid

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-ATP8B1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Catalytic component of a P4-ATPase flippase complex which catalyzes the hydrolysis of ATP coupled to the transport of aminophospholipids from the outer to the inner leaflet of various membranes and ensures the maintenance of asymmetric distribution of phospholipids. Phospholipid translocation seems also to be implicated in vesicle formation and in uptake of lipid signaling molecules. May play a role in 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. In cooperation with ABCB4 may be involved in establishing integrity of the canalicular membrane thus protecting hepatocytes from bile salts. Together with TMEM30A is involved in uptake of the synthetic drug alkylphospholipid perifosine. Involved in the microvillus formation in polarized epithelial cells; the function seems to be independent from its flippase activity. Required for the preservation of cochlear hair cells in the inner ear. May act as cardiolipin transporter during inflammatory injury.

ELISA (EIA)

143,695 Da

ATPase phospholipid transporting 8B1

phospholipid-transporting ATPase IC

Phospholipid-transporting ATPase IC

ATPIC; FIC1; PFIC  [Similar Products]

AT8B1_HUMAN

This gene encodes a member of the P-type cation transport ATPase family, which belongs to the subfamily of aminophospholipid-transporting ATPases. The aminophospholipid translocases transport phosphatidylserine and phosphatidylethanolamine from one side of a bilayer to another. Mutations in this gene may result in progressive familial intrahepatic cholestasis type 1 and in benign recurrent intrahepatic cholestasis. [provided by RefSeq, Jul 2008]

ATP8B1: 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. Defects in ATP8B1 are the cause of progressive familial intrahepatic cholestasis type 1 (PFIC1); 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 *****hood. Defects in ATP8B1 are the cause of benign recurrent intrahepatic cholestasis type 1 (BRIC1); 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); 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. Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IV subfamily.

Protein type: Membrane protein, integral; Hydrolase; Membrane protein, multi-pass; EC 3.6.3.1; Transporter, ion channel; Transporter

Chromosomal Location of Human Ortholog: 18q21.31

Cellular Component: apical plasma membrane; endoplasmic reticulum; Golgi apparatus; plasma membrane

Molecular Function: phospholipid-translocating ATPase activity; protein binding

Biological Process: Golgi organization and biogenesis; multidrug transport; negative regulation of transcription, DNA-dependent; phospholipid translocation; regulation of microvillus biogenesis

Disease: Cholestasis, Benign Recurrent Intrahepatic, 1; Cholestasis, Intrahepatic, Of Pregnancy, 1; Cholestasis, Progressive Familial Intrahepatic, 1

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