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Titolo:
Uroporphyria in HFE mutant mice given 5-aminolevulinate: A new model of Fe-mediated porphyria cutanea tarda
Autore:
Sinclair, PR; Gorman, N; Walton, HS; Bement, WJ; Sinclair, JF; Gerhard, GS; Szakacs, JG; Andrews, NC; Levy, JE;
Indirizzi:
VA Med Ctr 151, White River Junction, VT 05009 USA VA Med Ctr 151 White River Junction VT USA 05009 r Junction, VT 05009 USA Dartmouth Med Sch, Dept Biochem, Hanover, NH USA Dartmouth Med Sch Hanover NH USA Med Sch, Dept Biochem, Hanover, NH USA Dartmouth Med Sch, Dept Pharmacol Toxicol, Hanover, NH USA Dartmouth Med Sch Hanover NH USA Dept Pharmacol Toxicol, Hanover, NH USA Dartmouth Med Sch, Dept Pathol, Hanover, NH USA Dartmouth Med Sch HanoverNH USA h Med Sch, Dept Pathol, Hanover, NH USA Vet Adm Med Ctr, Salt Lake City, UT 84148 USA Vet Adm Med Ctr Salt Lake City UT USA 84148 Salt Lake City, UT 84148 USA Childrens Hosp, Div Hematol Oncol, Boston, MA 02115 USA Childrens Hosp Boston MA USA 02115 iv Hematol Oncol, Boston, MA 02115 USA Howard Hughes Med Inst, Boston, MA 02115 USA Howard Hughes Med Inst Boston MA USA 02115 Med Inst, Boston, MA 02115 USA Harvard Univ, Sch Med, Dept Med, Boston, MA USA Harvard Univ Boston MA USA rvard Univ, Sch Med, Dept Med, Boston, MA USA Harvard Univ, Sch Med, Dept Pediat, Boston, MA 02115 USA Harvard Univ Boston MA USA 02115 h Med, Dept Pediat, Boston, MA 02115 USA Brigham & Womens Hosp, Div Hematol, Boston, MA 02115 USA Brigham & Womens Hosp Boston MA USA 02115 v Hematol, Boston, MA 02115 USA
Titolo Testata:
HEPATOLOGY
fascicolo: 2, volume: 33, anno: 2001,
pagine: 406 - 412
SICI:
0270-9139(200102)33:2<406:UIHMMG>2.0.ZU;2-U
Fonte:
ISI
Lingua:
ENG
Soggetto:
HEREDITARY HEMOCHROMATOSIS; DECARBOXYLASE ACTIVITY; C282Y MUTATION; HEPATITIS-C; INBRED MICE; IRON; ACID; GENE; DEFICIENCY; OXIDATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
47
Recensione:
Indirizzi per estratti:
Indirizzo: Sinclair, PR VA Med Ctr 151, White River Junction, VT 05009 USA VA Med Ctr151 White River Junction VT USA 05009 T 05009 USA
Citazione:
P.R. Sinclair et al., "Uroporphyria in HFE mutant mice given 5-aminolevulinate: A new model of Fe-mediated porphyria cutanea tarda", HEPATOLOGY, 33(2), 2001, pp. 406-412

Abstract

Porphyria cutanea tarda (PCT), a liver disease with skin lesions caused byexcess liver production of uroporphyrin (URO), is associated with consumption of alcoholic beverages or estrogens, and moderate iron overload. Recently, it has been shown that many PCT patients carry mutations in the HFE gene, which is responsible for hereditary hemochromatosis, Mice homozygous foreither the null mutation in the Hfe gene or the C282Y missense mutation rapidly accumulate hepatic parenchymal iron similar to patients with hemochromatosis. Here we investigated whether disruption of the murine Hfe gene would result in hepatic uroporphyria, Mice homozygous for the Hfe-null mutation accumulated high levels of hepatic URO when fed 5-aminolevulinate (ALA), Hfe (+/-) mice also accumulated hepatic URO when fed ALA, but at a much slower rate. The amount of accumulated URO in the null mutant mice was similarto that in wild-type mice treated with iron carbonyl in the diet, or injected with iron dextran, Iron in both wildtype and Hfe (+/-) mice was mostly in Kupffer cells. In contrast, Hfe (-/-) mice had considerable parenchymal iron deposition as well, in a pattern similar to that observed in wild-typemice treated with iron carbonyl, URO accumulation was accompanied by 84% and 33% decreases in hepatic uroporphyrinogen decarboxylase activities in Hfe (-/-) and Hfe (+/-) mice, respectively, No increases in CYP1A2, or other cytochrome P450s were detected in the Hfe-null mutant mice. We conclude that this experimental model of uroporphyria is a valid model for further investigations into the mechanism of PCT.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 15/08/20 alle ore 18:47:46