Gene/Proteome Database (LMPD)

LMPD ID
LMP001255
Gene ID
54670
Species
Mus musculus (Mouse)
Gene Name
ATPase, class I, type 8B, member 1
Gene Symbol
Atp8b1
Synonyms
AI451886; FIC1; Ic
Alternate Names
phospholipid-transporting ATPase IC; ATPase 8B1, p type; ATPase B1, class I; aminophospholipid translocase; familial intrahepatic cholestasis type I; probable phospholipid-transporting ATPase IC; P4-ATPase flippase complex alpha subunit ATP8B1
Chromosome
18
Map Location
18 E1|18
EC Number
3.6.3.1

Proteins

phospholipid-transporting ATPase IC
Refseq ID NP_001001488
Protein GI 80861460
UniProt ID Q148W0
mRNA ID NM_001001488
Length 1251
RefSeq Status VALIDATED
MSTERDSETTFDEESQPNDEVVPYSDDETEDELEDQGSTVEPEQNRVNREAEKKRETFRKDCTWQVKANDRKFHEQPHFMNTKFFCIKESKYASNAIKTYKYNGFTFLPMNLFEQFKRAANFYFLILLILQAIPQISTLAWYTTLVPLLLVLGITAIKDLVDDVARHKMDKEINNRTCEVIKDGRFKIIKWKDIQVGDVIRLKKNDFIPADILLLSSSEPNSLCYVETAELDGETNLKFKMALEITDQYLQIEDNLATFDGFIECEEPNNRLDKFTGTLFWKNQSFPLDADKILLRGCVIRNTDVCHGLVIFAGADTKIMKNSGKTRFKRTKIDYLMNYMVYTIFIVLILVSAGLAIGHAYWEAQVGNYSWYLYDGENATPSYRGFLNFWGYIIVLNTMVPISLYVSVEVIRLGQSHFINWDLQMYYAEKDTPAKARTTTLNEQLGQIHYIFSDKTGTLTQNIMTFKKCCINGTIYGDHRDASQHSHSKIELVDFSWNTFADGKLAFYDHYLIEQIQSGKEPEVRQFFFLLSICHTVMVDRIDGQINYQAASPDEGALVNAARNFGFAFLARTQNTITVSELGSERTYNVLAILDFNSDRKRMSIIVRTPEGSIRLYCKGADTVIYERLHRMNPTKQETQDALDIFASETLRTLCLCYKEIEEKEFTEWNNKFMAASVASSNRDEALDKVYEEIEKDLILLGATAIEDKLQDGVPETISKLAKADIKIWVLTGDKKETAENIGFACELLTEDTTICYGEDINSLLHTRMENQRNRGGVSAKFAPPVYEPFFPPGENRALIITGSWLNEILLEKKTKRSKILKLKFPRTEEERRMRSQSRRRLEEKKEQRQKNFVDLACECSAVICCRVTPKQKAMVVDLVKRYKKAITLAIGDGANDVNMIKTAHIGVGISGQEGMQAVMSSDYSFAQFRYLQRLLLVHGRWSYIRMCKFLRYFFYKNFAFTLVHFWYSFFNGYSAQTAYEDWFITLYNVLYSSLPVLLMGLLDQDVSDKLSLRFPGLYVVGQRDLLFNYKRFFVSLLHGVLTSMVLFFIPLGAYLQTVGQDGEAPSDYQSFAVTVASALVITVNFQIGLDTSYWTFVNAFSIFGSIALYFGIMFDFHSAGIHVLFPSAFQFTGTASNALRQPYIWLTIILTVAVCLLPVVAIRFLSMTIWPSESDKIQKHRKRLKAEEQWKRRQSVFRRGVSSRRSAYAFSHQRGYADLISSGRSIRKKRSPLDAIIADGTAEYRRTVES

Gene Information

Entrez Gene ID
54670
Gene Name
ATPase, class I, type 8B, member 1
Gene Symbol
Atp8b1
Species
Mus musculus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005794 IEA:UniProtKB-KW C Golgi apparatus
GO:0016324 IEA:Ensembl C apical plasma membrane
GO:0031526 IEA:Ensembl C brush border membrane
GO:0005783 IEA:UniProtKB-KW C endoplasmic reticulum
GO:0016021 IEA:UniProtKB-KW C integral component of membrane
GO:0032420 IDA:UniProtKB C stereocilium
GO:0005524 IEA:UniProtKB-KW F ATP binding
GO:1901612 IDA:UniProtKB F cardiolipin binding
GO:0019829 IEA:InterPro F cation-transporting ATPase activity
GO:0000287 IEA:InterPro F magnesium ion binding
GO:0004012 IEA:UniProtKB-EC F phospholipid-translocating ATPase activity
GO:0008206 IMP:MGI P bile acid metabolic process
GO:0006855 IEA:Ensembl P drug transmembrane transport
GO:0060119 IMP:UniProtKB P inner ear receptor cell development
GO:0045892 IEA:Ensembl P negative regulation of transcription, DNA-templated
GO:0032534 IEA:Ensembl P regulation of microvillus assembly
GO:0007605 IMP:UniProtKB P sensory perception of sound
GO:0021650 IMP:UniProtKB P vestibulocochlear nerve formation

REACTOME Pathway Links

REACTOME Pathway ID Description
5893745 Ion channel transport
5893744 Ion transport by P-type ATPases
5892775 Transmembrane transport of small molecules

Domain Information

InterPro Annotations

Accession Description
IPR008250 ATPase_P-typ_transduc_dom_A
IPR001757 Cation_transp_P_typ_ATPase
IPR023214 HAD-like domain
IPR023299 P-type ATPase, cytoplasmic domain N
IPR018303 P-type ATPase, phosphorylation site
IPR006539 P-type ATPase, subfamily IV

UniProt Annotations

Entry Information

Gene Name
ATPase, class I, type 8B, member 1
Protein Entry
AT8B1_MOUSE
UniProt ID
Q148W0
Species
Mouse

Comments

Comment Type Description
Catalytic Activity ATP + H(2)O + phospholipid(Side 1) = ADP + phosphate + phospholipid(Side 2).
Disruption Phenotype Mice have unimpaired bile secretion, and no liver damage, but show mild abnormalities including depressed weight at weaning and elevated serum bile salt levels. Do not suffer from jaundice or diarrhea and have normal serum bilirubin levels and normal liver enzyme activities, except for mildly elevated serum AST (aspartate aminotransferase) activity. Display unimpaired transhepatic bile salt transport and are resistant to bile salt-induced cholestasis. Upon bile salt feeding, demonstrate serum bile salt accumulation, hepatic injury and expansion of the systemic bile salt pool and this failure of bile salt homeostasis occurrs in the absence of any defect in hepatic bile secretion. {ECO:0000269|PubMed:14976163}.
Function 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. Plays a role in bile salt homeostasis. In cooperation with ABCB4 may be involved in establishing integrity of the canalicular membrane thus protecting hepatocytes from bile salts. 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. Required for the preservation of cochlear hair cells in the inner ear. According PubMed:20852622 is proposed to act as cardiolipin transporter during inflammatory injury; the function is questioned by PubMed:21475228. {ECO:0000269|PubMed:14976163, ECO:0000269|PubMed:19478059, ECO:0000269|PubMed:20852622, ECO:0000269|PubMed:21475228, ECO:0000269|PubMed:21820390}.
Similarity Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IV subfamily. {ECO:0000305}.
Subcellular Location Cell membrane {ECO:0000269|PubMed:20852622}; Multi-pass membrane protein {ECO:0000269|PubMed:20852622}. Apical cell membrane {ECO:0000250}. Cell projection, stereocilium {ECO:0000269|PubMed:20852622}. Endoplasmic reticulum {ECO:0000250}. Golgi apparatus {ECO:0000250}. Note=Exit from the endoplasmic reticulum requires the presence of TMEM30A or TMEM30B.
Subunit Component of a P4-ATPase flippase complex which consists of a catalytic alpha subunit and an accessory beta subunit. The probable flippase ATP8B1:TMEM30A complex can form an intermediate phosphoenzyme in vitro. Also interacts with beta subunit TMEM30B (By similarity). {ECO:0000250}.
Tissue Specificity Hepatocytes, bile duct, intestinal epithelial cells (cholangiocytes and ileocytes), and pancreatic acinar cells. {ECO:0000269|PubMed:14976163}.

Identical and Related Proteins

Unique RefSeq proteins for LMP001255 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
80861460 RefSeq NP_001001488 1251 phospholipid-transporting ATPase IC

Identical Sequences to LMP001255 proteins

Reference Database Accession Length Protein Name
GI:80861460 SwissProt Q148W0.2 1251 RecName: Full=Phospholipid-transporting ATPase IC; AltName: Full=ATPase class I type 8B member 1; AltName: Full=P4-ATPase flippase complex alpha subunit ATP8B1 [Mus musculus]

Related Sequences to LMP001255 proteins

Reference Database Accession Length Protein Name
GI:80861460 DBBJ BAE34046.1 1251 unnamed protein product [Mus musculus]
GI:80861460 GenBank AAR90342.1 1251 ATPase class I type 8B member 1 [Mus musculus]
GI:80861460 GenBank AAI17947.1 1251 ATPase, class I, type 8B, member 1 [Mus musculus]
GI:80861460 GenBank EDL09700.1 1259 ATPase, class I, type 8B, member 1 [Mus musculus]
GI:80861460 RefSeq NP_001099610.1 1259 phospholipid-transporting ATPase IC [Rattus norvegicus]
GI:80861460 RefSeq XP_006254904.1 1251 PREDICTED: phospholipid-transporting ATPase IC isoform X1 [Rattus norvegicus]