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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

Related topics

Related topics

7 relations

Broader (3)

Amino Acid Metabolism, Inborn ErrorsEnzyme DeficiencyRetinal Degeneration
Gyrate AtrophyHHH syndromeOAT geneOrnithine-oxo-acid aminotransferase

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2018
Highly Cited
2018
One Hundred Fifteen Cases of Pure Laparoscopic Living Donor Right Hepatectomy at a Single Center
Background The pure laparoscopic approach to donor hepatectomy is being taken more often. However, few centers perform pure… 
Review
2015
Review
2015
The hyperornithinemia–hyperammonemia-homocitrullinuria syndrome
BackgroundHyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive disorder of the urea… 
Highly Cited
2009
Highly Cited
2009
Identification of novel mutations in the SLC25A15 gene in hyperornithinemia‐hyperammonemia‐homocitrullinuria (HHH) syndrome: A clinical, molecular, and functional study
Hyperornithinemia‐hyperammonemia‐homocitrullinuria (HHH) syndrome is an autosomal recessive disorder of the urea cycle. With the… 
Review
2004
Review
2004
Arginases I and II: do their functions overlap?
Review
2002
Review
2002
Diagnosis and monitoring of inborn errors of metabolism using urease-pretreatment of urine, isotope dilution, and gas chromatography-mass spectrometry.
  • T. Kuhara
  • Journal of chromatography. B, Analytical…
  • 2002
  • Corpus ID: 1014398
Highly Cited
2001
Highly Cited
2001
Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
The authors report the clinical and molecular findings in eight patients with hyperornithinemia, hyperammonemia, and… 
2001
2001
Separation of free amino acids in human plasma by capillary electrophoresis with laser induced fluorescence: potential for emergency diagnosis of inborn errors of metabolism.
Review
2000
Review
2000
Fatal Hyperammonemia after Orthotopic Lung Transplantation
Hyperammonemia has been reported in association with a variety of disorders causing hepatic dysfunction: portosystemic… 
1988
1988
5-Fluoromethylornithine, an irreversible and specific inhibitor of L-ornithine:2-oxo-acid aminotransferase.
5-Fluoromethylornithine (5-FMOrn) is the first specific irreversible inhibitor of L-ornithine:2-oxoacid aminotransferase (OAT… 
Highly Cited
1982
Highly Cited
1982
A New Synaptosomal Biosynthetic Pathway of Glutamate and GABA from Ornithine and Its Negative Feedback Inhibition by GABA
Abstract: In sonicates of mouse brain synaptosomes, we demonstrated that γ‐aminobutyric acid (GABA) can be formed from l… 
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