ATP8B1 sirna

ATP8B1 siRNA (Human)

Cat. Num. AAA8220296

• Gene Names: ATP8B1; BRIC; FIC1; ICP1; PFIC; ATPIC; PFIC1
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: ATP8B1; ATP8B1 siRNA (Human); ATPIC; FIC1; PFIC; Probable phospholipid-transporting ATPase IC; ATPase class I type 8B member 1; Familial intrahepatic cholestasis type 1; ATP8B1 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: ATP8B1 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 1251

• Applicable Applications for ATP8B1 sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human ATP8B1 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for ATP8B1 sirna

siRNA to inhibit ATP8B1 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 5031697
• NCBI GeneID: 5205
• NCBI Accession #: NP_005594.1
• NCBI GenBank Nucleotide #: NM_005603.4
• UniProt Accession #: O43520; Q9BTP8
• Molecular Weight: 143,695 Da
• NCBI Official Full Name: phospholipid-transporting ATPase IC
• NCBI Official Synonym Full Names: ATPase, aminophospholipid transporter, class I, type 8B, member 1
• NCBI Official Symbol: ATP8B1
• NCBI Official Synonym Symbols: BRIC; FIC1; ICP1; PFIC; ATPIC; PFIC1
• NCBI Protein Information: phospholipid-transporting ATPase IC
• UniProt Protein Name: Phospholipid-transporting ATPase IC
• Protein Family: Phospholipid-transporting ATPase
• UniProt Gene Name: ATP8B1
• UniProt Synonym Gene Names: ATPIC; FIC1; PFIC
• UniProt Entry Name: AT8B1_HUMAN

NCBI Description

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]

Uniprot Description

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 adulthood. 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: Hydrolase; Transporter, ion channel; Membrane protein, multi-pass; EC 3.6.3.1; Transporter; Membrane protein, integral

Chromosomal Location of Human Ortholog: 18q21.31

Cellular Component: Golgi apparatus; stereocilium; integral to plasma membrane; endoplasmic reticulum; brush border membrane; apical plasma membrane; plasma membrane

Molecular Function: phospholipid-translocating ATPase activity; protein binding; magnesium ion binding; ATP binding

Biological Process: bile acid and bile salt transport; phospholipid translocation; sensory perception of sound; vestibulocochlear nerve formation; inner ear receptor cell development; bile acid metabolic process; multidrug transport; negative regulation of transcription, DNA-dependent; Golgi organization and biogenesis; transmembrane transport; regulation of microvillus biogenesis

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