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HHH syndrome

Known as: HHH, Triple H Syndrome, Hyperornithinemia-hyperammonemia-homocitrullinuria 
An autosomal recessive disorder caused by mutation(s) in the SLC25A15 gene, encoding mitochondrial ornithine transporter 1. The condition is… 
National Institutes of Health

Related topics

Related topics

25 relations
Abnormal pyramidal signsAcute encephalopathyAcute hepatitisAmino acidemia disorder suspected:Prid:Pt:Bld.dot:Nom

Broader (2)

OrnithineUrea Cycle Disorders, Inborn

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2015
Highly Cited
2015
Patterns of Walkability, Transit, and Recreation Environment for Physical Activity.
2006
2006
HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation
SummaryWe report a 3-year-old Italian patient with the hyperornithinaemia, hyperammonaemia, homocitrullinuria (HHH) syndrome who… 
2006
2006
A Foot-Based Reanalysis of Edge-inTonal Phenomena in Bambara
Zoll cites the case of Kukuya, with trisyllabic patterns of LLL, HHH, HLL, LLH and LHL. The absence of *HHL and LHH motivates the… 
Highly Cited
2004
Highly Cited
2004
Supramolecular recognition of heteropairs of lanthanide ions: a step toward self-assembled bifunctional probes.
Three unsymmetrical ditopic hexadentate ligands coded for the recognition of trivalent lanthanide ions have been synthesized, L… 
Highly Cited
2002
Highly Cited
2002
Effect of maternal undernutrition on fetal testicular steroidogenesis during the CNS androgen-responsive period in male sheep fetuses.
The aim of this study was to determine the effects of maternal undernutrition, applied during physiologically relevant stages of… 
Highly Cited
1999
Highly Cited
1999
Hyperinsulinism-hyperammonemia syndrome caused by mutant glutamate dehydrogenase accompanied by novel enzyme kinetics
Abstract Hyperinsulinism-hyperammonemia syndrome (HHS) is a recently identified genetic disorder characterized by… 
1988
1988
Biochemical analysis of decreased ornithine transport activity in the liver mitochondria from patients with hyperornithinemia, hyperammonemia and homocitrullinuria.
1982
1982
Defective ornithine metabolism in cultured skin fibroblasts from patients with the syndrome of hyperornithinemia, hyperammonemia and homocitrullinuria.
Highly Cited
1980
Highly Cited
1980
Power motivation, stress and physical illness.
College students reporting a large number of life change events in the past six months also reported significantly more frequent… 
1973
1973
The role of brain monoamines and pineal indoles in the secretion of gonadotrophins and gonadotrophin-releasing factors.
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