Overview

Variant ID 3551
Entrez Gene ID 8647
Gene ABCB11 (GeneCards)
Location hg19 2:169890984-169890984
hg38 2:169034474-169034474
Disease
Method HiSeq X Ten
Mutation(HGVS format) NC_000002.11:g.169890984 C>T (Genome Assembly: GRCh37)

Other information

Exon or Intron NA
Position in protein NA
Amino acid changes in protein NA > NA
Position in cDNA NA
Changes in cDNA NA > NA
mRNA accession NA
mRNA length NA
Reference length 243199373

Annotations and predictions

MAF in gnomAD genome (version 2.0.1) 0
EIGEN score 0.1773
CADD Raw score (version 1.3) 0.15136 (Deleterious)
FATHMM raw prediction score 0.17379 (Tolerated)
Deleterious probability by DeFine 0.4551 (Neutral)
Entrez Gene ID 8647 (NCBI Gene)
Official Gene Symbol ABCB11 (GeneCards)
Number of variants in ABCB11 in this database 3 (view all the variants)
Full name ATP binding cassette subfamily B member 11
Band 2q31.1
Other IDs Vega: OTTHUMG00000154039
OMIM: 603201
HGNC: HGNC:42
Ensembl: ENSG00000073734
Other names BSEP, PGY4, SPGP, ABC16, BRIC2, PFIC2, PFIC-2
Summary The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is the major canalicular bile salt export pump in man. Mutations in this gene cause a form of progressive familial intrahepatic cholestases which are a group of inherited disorders with severe cholestatic liver disease from early infancy. [provided by RefSeq, Jul 2008]

Publication #1: 29217584

Pubmed ID 29217584
Title Aging and neurodegeneration are associated with increased mutations in single human neurons.
Journal Science
Publication date 2018.02
Disease Cockayne syndrome Xeroderma Pigmentosum
Number of cases Male cases: 3; Female cases: 6; cases of unknown sex: 15;