PMID: 8593585Oct 30, 1995Paper

Behavioral pathology induced by repeated systemic injections of 3-nitropropionic acid mimics the motoric symptoms of Huntington's disease

Brain Research
C V BorlonganP R Sanberg

Abstract

Huntington's disease is a progressive neurodegenerative disorder associated with severe degeneration of basal ganglia neurons, especially the intrinsic neurons of the striatum, and characterized by involuntary abnormal choreiform movements and progressive dementia. With the discovery of the gene underlying HD, genetic therapy may be the next logical step towards finding a cure, but no such treatment is currently available. Animal models that closely mimic the neurobiological and clinical symptoms of the disease may offer an alternative approach for the development of new therapies. We report that systemic administration of 3-nitropropionic acid, an inhibitor of the mitochondrial citric acid cycle, results in a progressive locomotor deterioration resembling that of HD. We further demonstrate that manipulating the time course of 3-nitropropionic acid injections leads to sustained hyperactivity (early HD) or hypoactivity (advanced HD). These data suggest that this animal model can be used to test experimental treatments for HD across different stages of the disease.

Citations

Sep 2, 1998·Current Opinion in Neurology·A Feigin
Aug 2, 2002·Current Opinion in Neurology·Andrew Feigin, Dennis Zgaljardic
Nov 24, 1999·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·F WiegandU Dirnagl
Sep 2, 2017·The Journal of Membrane Biology·Anita WnętrzakPatrycja Dynarowicz-Łątka
Jul 1, 1997·Brain Pathology·C L WellingtonM R Hayden
Sep 29, 2000·Movement Disorders : Official Journal of the Movement Disorder Society·R J CarterA J Morton
Jan 5, 2002·Expert Opinion on Investigational Drugs·C M Rodrigues, C J Steer
Apr 15, 2003·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·M SuttonBrown, O Suchowersky
Feb 13, 2001·British Journal of Pharmacology·D PubillA Camins
Jul 1, 2008·The European Journal of Neuroscience·Joana M Gil, Ana Cristina Rego
Aug 6, 1999·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·P H ReddyD A Tagle
Sep 1, 2008·Interdisciplinary Toxicology·Russel J ReiterDun-Xian Tan
Jun 29, 2012·Neurotoxicity Research·Cristiani F BortolattoCristina W Nogueira
Jan 12, 2019·Neurotoxicity Research·Musthafa Mohamed EssaMohammed Akbar
Mar 26, 2010·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Isaac TúnezAbel Santamaría
Mar 7, 2003·Human Molecular Genetics·Stephan von HörstenOlaf Riess
Jan 31, 2012·Neurology Research International·Tatyana VagnerAlexandre Mouravlev
Mar 7, 2021·International Journal of Molecular Sciences·Seulah LeeJaewon Lee
Feb 1, 2008·Progress in Neuro-psychopharmacology & Biological Psychiatry·Ashish DhirS K Kulkarni

❮ Previous
Next ❯

Related Concepts

Related Feeds

Basal Ganglia

Basal Ganglia are a group of subcortical nuclei in the brain associated with control of voluntary motor movements, procedural and habit learning, emotion, and cognition. Here is the latest research.