Switch to
Predicate | Object |
---|---|
rdf:type | |
lifeskim:mentions | |
pubmed:issue |
5
|
pubmed:dateCreated |
1995-6-2
|
pubmed: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.
|
pubmed:language |
eng
|
pubmed:journal | |
pubmed:citationSubset |
IM
|
pubmed:chemical | |
pubmed:status |
MEDLINE
|
pubmed:month |
May
|
pubmed:issn |
0270-9139
|
pubmed:author | |
pubmed:issnType |
Print
|
pubmed:volume |
21
|
pubmed:owner |
NLM
|
pubmed:authorsComplete |
Y
|
pubmed:pagination |
1405-12
|
pubmed:dateRevised |
2006-11-15
|
pubmed:meshHeading |
pubmed-meshheading:7537713-Animals,
pubmed-meshheading:7537713-Cell Transplantation,
pubmed-meshheading:7537713-Histidine,
pubmed-meshheading:7537713-Histidine Ammonia-Lyase,
pubmed-meshheading:7537713-Hybridization, Genetic,
pubmed-meshheading:7537713-Immunohistochemistry,
pubmed-meshheading:7537713-Liver,
pubmed-meshheading:7537713-Mice,
pubmed-meshheading:7537713-Mice, Inbred C57BL,
pubmed-meshheading:7537713-Mice, Inbred Strains,
pubmed-meshheading:7537713-Staining and Labeling
|
pubmed:year |
1995
|
pubmed:articleTitle |
Histidinemia in mice: a metabolic defect treated using a novel approach to hepatocellular transplantation.
|
pubmed:affiliation |
Department of Medicine, Royal Postgraduate Medical School, London, England.
|
pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|