Ornithine In Vivo Administration Disrupts Redox Homeostasis and Decreases Synaptic Na(+), K (+)-ATPase Activity in Cerebellum of Adolescent Rats: Implications for the Pathogenesis of Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH) Syndrome

Cellular and Molecular Neurobiology
Angela ZanattaMoacir Wajner

Abstract

Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is an inborn error of metabolism caused by a defect in the transport of ornithine (Orn) into mitochondrial matrix leading to accumulation of Orn, homocitrulline (Hcit), and ammonia. Affected patients present a variable clinical symptomatology, frequently associated with cerebellar symptoms whose pathogenesis is poorly known. Although in vitro studies reported induction of oxidative stress by the metabolites accumulating in HHH syndrome, so far no report evaluated the in vivo effects of these compounds on redox homeostasis in cerebellum. Therefore, the present work was carried out to investigate the in vivo effects of intracerebellar administration of Orn and Hcit on antioxidant defenses (reduced glutathione concentrations and the activities of superoxide dismutase, catalase, glutathione peroxidase, glutathione reductase, and glucose-6-phosphate dehydrogenase), lipid oxidation (malondialdehyde concentrations), as well as on the activity of synaptic Na(+), K(+)-ATPase, an enzyme highly vulnerable to free radical attack, in the cerebellum of adolescent rats. Orn significantly increased malondialdehyde levels and the activities of all antioxidant enzymes, and reduced...Continue Reading

References

Nov 1, 1992·Journal of Pediatric Gastroenterology and Nutrition·L SmithE G Seidman
Mar 1, 1992·Journal of Neurochemistry·E Satoh, Y Nakazato
Sep 1, 1986·Analytical Biochemistry·K M ChanK D Junger
Jan 1, 1985·Methods in Enzymology·I Carlberg, B Mannervik
Oct 1, 1986·Physiology & Behavior·J P Kroon, A L Riley
Jul 1, 1984·Journal of Neurochemistry·S F Leong, J B Clark
Jan 1, 1984·Methods in Enzymology·H Aebi
Jan 1, 1981·Methods in Enzymology·A Wendel
May 1, 1994·Progress in Neurobiology·M Erecińska, I A Silver
Dec 24, 1997·Annals of the New York Academy of Sciences·E KurellaA Boldyrev
Aug 17, 2000·Nitric Oxide : Biology and Chemistry·P Muriel, G Sandoval
May 23, 2001·Archives of Biochemistry and Biophysics·P EvelsonE A Lissi
Jan 30, 2002·Pediatric Neurology·Takeshi MiyamotoSeiichi Tsujino
Jul 30, 2003·Brain Research·Elena KosenkoVicente Felipo
Mar 31, 2004·Molecular Genetics and Metabolism·Andrea L Gropman, Mark L Batshaw
Aug 18, 2004·Progress in Neurobiology·Maria ErecinskaIan A Silver
Sep 21, 2004·Journal of Bioenergetics and Biomembranes·M D NorenbergA R Jayakumar
Feb 4, 2005·BMC Neuroscience·Chad A DickeyDave Morgan
Apr 15, 2008·Biochimica Et Biophysica Acta·Ferdinando Palmieri
Jun 20, 2008·Molecular Genetics and Metabolism·Mendel TuchmanUNKNOWN Additional members of Urea Cycle Disorders Consortium of the Rare Diseases Clinical Research Network
Dec 11, 2008·Acta Neurochirurgica. Supplement·T LekicJ H Zhang
Aug 18, 2009·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Alexandre Umpierrez AmaralMoacir Wajner
Dec 17, 2009·Acta Neuropathologica·Michael V Sofroniew, Harry V Vinters
Mar 9, 2010·Molecular Genetics and Metabolism·Andrea Gropman
Mar 17, 2010·Molecular Genetics and Metabolism·Olivier Braissant

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