| First Author | Selden C | Year | 1995 |
| Journal | Hepatology | Volume | 21 |
| Issue | 5 | Pages | 1405-12 |
| PubMed ID | 7537713 | Mgi Jnum | J:292668 |
| Mgi Id | MGI:6436155 | Doi | 10.1002/hep.1840210526 |
| Citation | Selden C, et al. (1995) Histidinemia in mice: a metabolic defect treated using a novel approach to hepatocellular transplantation. Hepatology 21(5):1405-12 |
| abstractText | Histidinemia in mice and in humans is an autosomal recessive disorder of 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 in a 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 the first to show complete correction of a defective biochemical phenotype achieved by hepatocellular transplantation. |
