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Titolo:
Correction of liver disease following transplantation of normal rat hepatocytes into long-evans cinnamon rats modeling Wilson's disease
Autore:
Irani, AN; Malhi, H; Slehria, S; Gorla, GR; Volenberg, I; Schilsky, ML; Gupta, S;
Indirizzi:
Yeshiva Univ Albert Einstein Coll Med, Dept Med, Bronx, NY 10461 USA Yeshiva Univ Albert Einstein Coll Med Bronx NY USA 10461 nx, NY 10461 USA Yeshiva Univ Albert Einstein Coll Med, Dept Radiat Oncol, Bronx, NY 10461 USA Yeshiva Univ Albert Einstein Coll Med Bronx NY USA 10461 nx, NY 10461 USA Yeshiva Univ Albert Einstein Coll Med, Marion Bessin Liver Res Ctr, Bronx,NY 10461 USA Yeshiva Univ Albert Einstein Coll Med Bronx NY USA 10461 onx,NY 10461 USA Yeshiva Univ Albert Einstein Coll Med, Comprehens Canc Res Ctr, Bronx, NY 10461 USA Yeshiva Univ Albert Einstein Coll Med Bronx NY USA 10461 nx, NY 10461 USA Yeshiva Univ Albert Einstein Coll Med, Gen Clin Res Ctr, Bronx, NY 10461 USA Yeshiva Univ Albert Einstein Coll Med Bronx NY USA 10461 nx, NY 10461 USA St Lukes Roosevelt Med Ctr, Natl Ctr Study Wilsons Dis, New York, NY 10019USA St Lukes Roosevelt Med Ctr New York NY USA 10019 s, New York, NY 10019USA
Titolo Testata:
MOLECULAR THERAPY
fascicolo: 3, volume: 3, anno: 2001,
pagine: 302 - 309
SICI:
1525-0016(200103)3:3<302:COLDFT>2.0.ZU;2-L
Fonte:
ISI
Lingua:
ENG
Soggetto:
MOUSE-LIVER; DIFFERENTIATION; REPOPULATION; REPLACEMENT; INTEGRATION; POLYPLOIDY; COPPER; CELLS;
Keywords:
cell; treatment; ATP 7B; copper; bile;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
23
Recensione:
Indirizzi per estratti:
Indirizzo: Gupta, S Yeshiva Univ Albert Einstein Coll Med, Dept Med, 1300 Morris Pk Ave,Ullmann 625, Bronx, NY 10461 USA Yeshiva Univ Albert Einstein Coll Med 1300 Morris Pk Ave,Ullmann 625 Bronx NY USA 10461
Citazione:
A.N. Irani et al., "Correction of liver disease following transplantation of normal rat hepatocytes into long-evans cinnamon rats modeling Wilson's disease", MOL THER, 3(3), 2001, pp. 302-309

Abstract

To establish the efficacy of cell therapy in Wilson's disease, we used theLong-Evans Cinnamon (LEC) rat model with atp7b gene mutation and copper toxicosis. Several groups of LEC rats were established, including animals pretreated with retrorsine to exacerbate copper toxicosis and inhibit proliferation in native hepatocytes followed by partial hepatectomy to promote liver repopulation. Hepatocytes from normal, syngeneic LEA rats were transplanted intrasplenically. Animal survival, biliary copper excretion, and hepaticcopper were determined. The magnitude of liver repopulation was demonstrated by measuring serum ceruloplasmin and hepatic atp7b mRNA. Long-term survival in LEC rats treated with retrorsine, partial hepatectomy, and cell transplantation was up to 90%, whereas fewer than 10% of animals pretreated with retrorsine, without cell therapy, survived, P < 0.001. Liver repopulationoccurred gradually after cell transplantation, ranging from <25% at 6 weeks, 26 to 40% at 4 months, and 74 to 100% at 6 months or beyond. Liver repopulation restored biliary copper excretion capacity and lowered liver copperlevels. Remarkably, liver histology was completely normal in LEC rats withextensive liver repopulation, compared with widespread megalocytosis, apoptosis, oval cell proliferation, and cholangiofibrosis in untreated animals. These data indicate that liver repopulation with functionally intact cellscan reverse pathophysiological perturbations and cure Wilson's disease.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/06/20 alle ore 00:30:59