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Hyperornithinemia

Known as: Ornithine Ketoacid Aminotransferase Deficiency, Ornithine Delta Aminotransferase Deficiency, Deficiency, OAT 
Increased concentration of ornithine in the blood. [HPO:probinson]
National Institutes of Health

Papers overview

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Review
2015
Review
2015
BackgroundHyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive disorder of the urea… Expand
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Review
2004
Review
2004
Arginase, often perceived solely as the last of the now six enzymes of the urea cycle, exists in two forms and has a broad tissue… Expand
Highly Cited
2001
Highly Cited
2001
The authors report the clinical and molecular findings in eight patients with hyperornithinemia, hyperammonemia, and… Expand
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Review
2000
Review
2000
Hyperammonemia has been reported in association with a variety of disorders causing hepatic dysfunction: portosystemic… Expand
1988
1988
5-Fluoromethylornithine (5-FMOrn) is the first specific irreversible inhibitor of L-ornithine:2-oxoacid aminotransferase (OAT… Expand
Highly Cited
1982
Highly Cited
1982
Abstract: In sonicates of mouse brain synaptosomes, we demonstrated that γ‐aminobutyric acid (GABA) can be formed from l… Expand
Highly Cited
1980
Highly Cited
1980
  • I. Sipilä
  • Biochimica et biophysica acta
  • 1980
  • Corpus ID: 980840
The inhibitory effect of ornithine on L-arginine:glycine amidinotransferase (EC 2.1.4.1) was studied in crude rat kidney… Expand
Highly Cited
1980
Highly Cited
1980
Four patients with gyrate atrophy of the choroid and retina were studied, all of whom exhibited the hyperornithinemia… Expand
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Highly Cited
1979
Highly Cited
1979
We studied 21 patients with gyrate atrophy of the choroid and retina and hyperornithinemia. Although the patients were not weak… Expand
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Highly Cited
1975
Highly Cited
1975
A case of gyrate atrophy of the choroid and retina and hyperornithinemia in a 28-year-old man was subjected to extensive clinical… Expand
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