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Article

Decreased hyperammonaemia and orotic aciduria due to inactivation of ornithine aminotransferase in mice with a hereditary abnormal ornithine carbamoyltransferase.

Journal of inherited metabolic disease - 1 Jan 1994

Seiler N, Grauffel C, Daune-Anglard G, Sarhan S, Knödgen B

Abstract excerpt

Mice with the X-chromosomal sparse-fur (spf) mutation are an animal model of some hereditary deficiencies of ornithine carbamoyltransferase (OCT) in man. Orotic aciduria and hyperammonaemia are the most conspicuous metabolic changes in these diseases. Selective inactivation of ornithine aminotransferase (OAT) by 5-fluoromethylornithine raises endogenous ornithine concentrations so that citrulline formation is...

Topics

Amino Acids
Ammonia
Animals
Behavior, Animal
Female
Male
Metabolism, Inborn Errors
Mice
Mice, Mutant Strains
Mutation
Ornithine

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Ornithine Carbamoyltransferase

Ornithine-Oxo-Acid Transaminase

Orotic Acid

Source

Identifiers

PMID: 7707692
DOI: 10.1007/BF00712011

Genes

OAT

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 29 Aug 2026. Prepared record publication-7707692-2026-08-29.1. Manifest publication-graph-cohort-2026-08-29.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 29 Aug 2026.

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Decreased hyperammonaemia and orotic aciduria due to inactivation of ornithine aminotransferase in mice with a hereditary abnormal ornithine carbamoyltransferase.PMID 7707692Read ↗

A new mouse model of mild ornithine transcarbamylase deficiency (spf-j) displays cerebral amino acid perturbations at baseline and upon systemic immune activation.Semantic proximity Read ↗

Free amino acid levels in serum and small intestine during the post-natal development of normal and sparse-fur mutant mice.Semantic proximity Read ↗

Reduced ornithine transcarbamylase activity does not impair ureagenesis in Otc(spf-ash) mice.Semantic proximity Read ↗

Ectopic correction of ornithine transcarbamylase deficiency in sparse fur mice.Semantic proximity Read ↗

Normalization of hair growth in sparse fur-abnormal skin and hair (SPF-ASH) mice by introduction of the rat ornithine transcarbamylase (OTC) gene.Semantic proximity Read ↗

Breeding experiments to combine the X-linked sparse-fur (spf) mutation with the autosomal recessive BALB/cByJ strain: testing the biochemical phenotype of double-mutant mice as a model for ammonia: fatty acyl CoA synergism.Semantic proximity Read ↗

Ex Vivo Enteroids Recapitulate In Vivo Citrulline Production in Mice.Semantic proximity Read ↗

The Mechanism of Hyperammonemia Triggered by Corticosteroid Administration in Late-Onset Ornithine Transcarbamylase DeficiencySemantic proximity Read ↗

Overexpression of ornithine aminotransferase: consequences on amino acid homeostasis.Semantic proximity Read ↗

Mice lacking ornithine-delta-aminotransferase have paradoxical neonatal hypoornithinaemia and retinal degeneration.Semantic proximity Read ↗

Mice deficient in the urea-cycle enzyme, carbamoyl phosphate synthetase I, die during the early neonatal period from hyperammonemia.Semantic proximity Read ↗

Hyperornithinaemia- hyperammonaemia- homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporterSemantic proximity Read ↗

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