Research ReportDual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia–hyperammonemia–homocitrullinuria syndrome
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Research highlights
►Ornithine (Orn) and homocitrulline (Hcit) accumulate in HHH syndrome. ►Affected patients present neurological symptoms whose pathogenesis is poorly known. ►Orn and Hcit provoke oxidative damage and reduce antioxidant defenses in the brain. ►Orn and Hcit inhibit major pathways of energy production in the brain. ►These pathomechanisms may be involved with the neuropathology of HHH syndrome.
Abbreviations
HHH
hyperornithinemia–hyperammonemia–homocitrullinuria
Orn
ornithine
Hcit
homocitrulline
ICV
intracerebroventricular
Keywords
Ornithine
Homocitrulline
Hyperornithinemia–hyperammonemia–homocitrullinuria syndrome
Oxidative stress
Energy metabolism
Cerebral cortex
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