PMID: 7537713May 1, 1995Paper

Histidinemia in mice: a metabolic defect treated using a novel approach to hepatocellular transplantation

Hepatology : Official Journal of the American Association for the Study of Liver Diseases
C SeldenH J Hodgson

Abstract

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 histid...Continue Reading

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Citations

Feb 7, 2001·The Journal of Gene Medicine·S GuptaP Novikoff
Mar 10, 2001·Clinics in Liver Disease·V L NgJ A Bezerra
Jun 1, 2007·Lab on a Chip·Eric GottwaldKarl-Friedrich Weibezahn
Jun 18, 2003·Transplantation·Ekaterine BerishviliZurab Kakabadze
May 26, 2004·Transplant Immunology·Clare Selden, Humphrey Hodgson
May 26, 2004·Transplant Immunology·Sanjeev GuptaDaniel Benten
Apr 9, 2010·The Journal of Investigative Dermatology·Joachim W FluhrKenneth R Feingold
Feb 1, 2011·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Humphrey Hodgson, Clare Selden
Aug 17, 2005·Journal of Thrombosis and Haemostasis : JTH·V KumaranS Gupta
Sep 22, 2009·Journal of Internal Medicine·E FitzpatrickA Dhawan
Jul 30, 2010·Journal of Hepatology·Kyle A SoltysIra J Fox

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