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Titolo:
BSEP: Function and role in progressive familial intrahepatic cholestasis
Autore:
Thompson, R; Strautnieks, S;
Indirizzi:
Univ London Kings Coll Hosp, Guys Kings & St Thomas Sch Med, Dept Child Hlth, London SE5 9RS, England Univ London Kings Coll Hosp London England SE5 9RS ndon SE5 9RS, England
Titolo Testata:
SEMINARS IN LIVER DISEASE
fascicolo: 4, volume: 21, anno: 2001,
pagine: 545 - 550
SICI:
0272-8087(2001)21:4<545:BFARIP>2.0.ZU;2-9
Fonte:
ISI
Lingua:
ENG
Soggetto:
SALT EXPORT PUMP; HEPATOCYTE CANALICULAR MEMBRANE; BILE-SALT; P-GLYCOPROTEIN; BYLER-DISEASE; SISTER GENE; LIVER; TRANSPORT; ATP; HETEROGENEITY;
Keywords:
liver; hepatocyte; hepatobiliary; transport; ABC proteins;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
32
Recensione:
Indirizzi per estratti:
Indirizzo: Thompson, R Univ London Kings Coll Hosp, Guys Kings & St Thomas Sch Med, Dept Child Hlth, Denmark Hill, London SE5 9RS, England Univ London Kings Coll Hosp Denmark Hill London England SE5 9RS
Citazione:
R. Thompson e S. Strautnieks, "BSEP: Function and role in progressive familial intrahepatic cholestasis", SEM LIV DIS, 21(4), 2001, pp. 545-550

Abstract

Secretion of bile acids is the major driving force for bile flow in mammals. The recently described adenosine triphosphate (ATP)-dependent bile acid transporter, bile salt export pump (BSEP), formerly, called sister of p-glycoprotein, is responsible for active transport of bile acids across the hepatocyte canalicular membrane into bile. It is now recognized that mutationsin the gene encoding this protein (ABCB11) are responsible for a subgroup of infants and children with progressive familial cholestasis (PFIC-2), a cholestatic disorder causing extreme pruritus, growth failure, and progression to cirrhosis in the first decade of life. Understanding the structure and function of BSEP has improved our understanding of the mechanisms underlying bile secretion. Determining genotype/phenotype relationships in patients with mutations in this gene are currently ongoing.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 07/07/20 alle ore 15:39:20