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Titolo:
HISTIDINEMIA IN MICE - A METABOLIC DEFECT TREATED USING A NOVEL-APPROACH TO HEPATOCELLULAR TRANSPLANTATION
Autore:
SELDEN C; CALNAN D; MORGAN N; WILCOX H; CARR E; HODGSON HJF;
Indirizzi:
ROYAL POSTGRAD MED SCH,DEPT MED,GASTROENTEROL UNIT,DUCANE RD LONDON W12 0NN ENGLAND ST HELIERS HOSP,DEPT CHEM PATHOL CARSHALTON SURREY ENGLAND
Titolo Testata:
Hepatology
fascicolo: 5, volume: 21, anno: 1995,
pagine: 1405 - 1412
SICI:
0270-9139(1995)21:5<1405:HIM-AM>2.0.ZU;2-F
Fonte:
ISI
Lingua:
ENG
Soggetto:
INTRASPLENIC HEPATOCYTE TRANSPLANTATION; MICROCARRIER-ATTACHED HEPATOCYTES; LIVER-CELL TRANSPLANTATION; RECEPTOR-DEFICIENT RABBITS; ENZYME DEFICIENCY; GUNN-RATS; MICROENCAPSULATED HEPATOCYTES; GENE-EXPRESSION; DENDRITIC CELLS; DISEASE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
59
Recensione:
Indirizzi per estratti:
Citazione:
C. Selden et al., "HISTIDINEMIA IN MICE - A METABOLIC DEFECT TREATED USING A NOVEL-APPROACH TO HEPATOCELLULAR TRANSPLANTATION", Hepatology, 21(5), 1995, pp. 1405-1412

Abstract

Histidinemia in mice and in humans is an autosomal recessive disorderof histidine metabolism that leads to high-histidine levels in both plasma and urine and is caused by a lack of hepatic histidine-alpha-deaminase (histidase). WE! have used a novel approach to hepatocellular transplantation to effect a complete phenotypic cure of histidinemia ina mouse model. Mice lacking histidase were treated with isolated Liver cells (approximately 18 x 10(6) hepatocytes and 9 x 10(6) nonparenchymal cells) from histidase-competent donors transplanted into the peritoneum (active transplant group). Recipient mice showed a dramatic decrease, by more than 75%, in urinary histidine levels from day one throughout the course of the experiment, resulting in levels within the normal range for wild-type mice. In comparison, there was no change in urinary histidine levels in the control group of histidase-deficient mice treated with isolated liver cells from mice lacking histidase (statistical comparison between the two groups, P <.003, two-way ANOVA). Histologically, ectopic liver tissue was seen in the peritoneum in association with abdominal wall, pancreas, and peritoneal connective tissue; immunohistochemical evidence showed expression of histidase in the ectopic liver tissue in the active transplant group. This report is thefirst to show complete correction of a defective biochemical phenotype achieved by hepatocellular transplantation.

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Documento generato il 22/01/21 alle ore 13:19:21