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Titolo:
FIC1, the protein affected in two forms of hereditary cholestasis, is localized in the cholangiocyte and the canalicular membrane of the hepatocyte
Autore:
Eppens, EF; van Mil, SWC; de Vree, JM; Mok, KS; Juijn, JA; Elferink, RPJO; Berger, R; Houwen, RHJ; Klomp, LWJ;
Indirizzi:
Univ Utrecht, Med Ctr, Dept Pediat Gastroenterol, NL-3584 AE Utrecht, Netherlands Univ Utrecht Utrecht Netherlands NL-3584 AE 3584 AE Utrecht, Netherlands Univ Amsterdam, Acad Med Ctr, Dept Expt Hepatol, NL-1105 AZ Amsterdam, Netherlands Univ Amsterdam Amsterdam Netherlands NL-1105 AZ Z Amsterdam, Netherlands Univ Utrecht, Med Ctr, Lab Metab Dis, NL-3584 AE Utrecht, Netherlands UnivUtrecht Utrecht Netherlands NL-3584 AE 3584 AE Utrecht, Netherlands
Titolo Testata:
JOURNAL OF HEPATOLOGY
fascicolo: 4, volume: 35, anno: 2001,
pagine: 436 - 443
SICI:
0168-8278(200110)35:4<436:FTPAIT>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
FAMILIAL INTRAHEPATIC CHOLESTASIS; BILE-ACID TRANSPORTER; P-TYPE ATPASES; BENIGN RECURRENT; HEPATOBILIARY TRANSPORT; RAT CHOLANGIOCYTES; PLASMA-MEMBRANE; BYLER DISEASE; LIVER; GENE;
Keywords:
FIC1; intrahepatic cholestasis; liver; canalicular membrane; PFIC1; benign recurrent intrahepatic cholestasis; hepatobiliary transport; P-type ATPase;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
32
Recensione:
Indirizzi per estratti:
Indirizzo: Klomp, LWJ Univ Utrecht, Med Ctr, Dept Pediat Gastroenterol, Room KC02-069, NL-3584 AE Utrecht, Netherlands Univ Utrecht Room KC02-069 Utrecht Netherlands NL-3584 AE ands
Citazione:
E.F. Eppens et al., "FIC1, the protein affected in two forms of hereditary cholestasis, is localized in the cholangiocyte and the canalicular membrane of the hepatocyte", J HEPATOL, 35(4), 2001, pp. 436-443

Abstract

Background/Aims: FIC1 (familial intrahepatic cholestasis 1) is affected intwo clinically distinct forms of hereditary cholestasis, namely progressive familial intrahepatic cholestasis type I (PFIC1) and benign recurrent intrahepatic cholestasis. Here we examined the subcellular localization of this protein within the liver. Methods: Antibodies raised against different epitopes of human FIC1 were used for immunoblot analysis and immunohistochemical detection of FIC1. Results: Immunoblot analysis of intestine and liver tissue extracts from human, rat and mouse origin indicated that the antibodies raised against FIC1 specifically detected FIC1 as a 140-kDa protein. In the liver homogenate of a PFIC1 patient, FIC1 could not be detected. Analysis of isolated rat liver membrane vesicles indicated that this protein is predominantly present in the canalicular membrane fraction. Immunohistochemical detection of the protein in liver sections confirmed that FIC1 was present in the canalicular membrane, whereas no staining was observed in the PFIC1 patients liver. Double label immunofluorescence of murine liver revealed that FIC1 colocalized xith cytokeratin 7 in cholangiocytes. Conclusions: The localization of FIC1 in the canalicular membrane and cholangiocytes suggests that it may directly or indirectly play a role in bile formation since mutations in FIC1 are associated with severe symptoms of cholestasis. (C) 2001 European Association for the Study of the Liver. Published by Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/11/20 alle ore 01:12:43