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Titolo:
Cholangiocyte biology and cystic fibrosis liver disease
Autore:
Feranchak, AP; Sokol, RJ;
Indirizzi:
Childrens Hosp, Dept Pediat, Sect Pediat Gastroenterol Hepatol & Nutr, Denver, CO 80218 USA Childrens Hosp Denver CO USA 80218 l Hepatol & Nutr, Denver, CO 80218 USA Univ Colorado, Hlth Sci Ctr, Denver, CO 80262 USA Univ Colorado Denver COUSA 80262 ado, Hlth Sci Ctr, Denver, CO 80262 USA
Titolo Testata:
SEMINARS IN LIVER DISEASE
fascicolo: 4, volume: 21, anno: 2001,
pagine: 471 - 488
SICI:
0272-8087(2001)21:4<471:CBACFL>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
TRANSMEMBRANE CONDUCTANCE REGULATOR; DUCT EPITHELIAL-CELLS; URSODEOXYCHOLIC ACID THERAPY; RECTIFYING CHLORIDE CHANNELS; INTRACELLULAR PH REGULATION; COMMON-BILE-DUCT; HEPATOBILIARY DISEASE; PORTAL-HYPERTENSION; RAT CHOLANGIOCYTES; BILIARY-CIRRHOSIS;
Keywords:
Cl- channel; cholestasis; bile; hepatocyte; secretion;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
165
Recensione:
Indirizzi per estratti:
Indirizzo: Feranchak, AP Childrens Hosp, Dept Pediat, Sect Pediat Gastroenterol Hepatol & Nutr, 1056 E 19th Ave,B290, Denver, CO 80218 USA Childrens Hosp 1056 E19th Ave,B290 Denver CO USA 80218 USA
Citazione:
A.P. Feranchak e R.J. Sokol, "Cholangiocyte biology and cystic fibrosis liver disease", SEM LIV DIS, 21(4), 2001, pp. 471-488

Abstract

Cystic fibrosis (CF) is one of the most common inherited diseases in the white population. The disease results from mutations in the gene for the cystic fibrosis transmembrane conductance regulator (CFTR). How this gene defect leads to the clinical manifestations of the disease, however, is not entirely clear. CFTR functions as a Cl- channel in the apical membrane of mostsecretory epithelia, including biliary epithelial cells, or cholangiocytes. In cholangiocytes, CFTR appears to be an important determinant of biliarysecretion and bile flow. Additionally, recent evidence suggests that CFTR regulates other membrane transporters, channels, and proteins. Improving life expectancy has led to an increasing recognition of hepatobiliary complications from CF. The true prevalence of CF liver disease is unknown, but mayaffect up to 17-25% of CF patients. Clinical manifestations include hepatic steatosis, neonatal cholestasis, focal nodular cirrhosis, multilobular cirrhosis, and biliary tract complications. Why only a subset of CF patients develops severe liver disease and others with the same genotype do not is one of the many scientific curiosities of this disease. This review focuses on the function of CFTR in cholangiocytes with emphasis on ductular bile formation as well as the clinical consequences of abnormal CFTR, namely CF-associated liver disease. Data on the pathogenesis, prevalence, clinical course, and treatment of CF liver disease will be reviewed.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 06:53:29