PMID: 9168265May 1, 1997Paper

3-Nitropropionic acid animal model and Huntington's disease

Neuroscience and Biobehavioral Reviews
C V BorlonganPaul R Sanberg

Abstract

Huntington's disease (HD) is a progressive neurodegenerative disorder associated with severe degeneration of basal ganglia neurons, especially the intrinsic neurons of the striatum, and characterized by progressive dementia and involuntary abnormal choreiform movements. Despite our increasing knowledge of the pathophysiology of HD, culminating with the discovery of the gene underlying HD, there has been no cure available to completely cease or reverse the progressive neurodegeneration and behavioral consequences of the disease. Animal models that closely mimic the neurobiological and clinical symptoms of the disease continue to offer alternative approaches for studying HD. Recently, we have reported that systemic administration of 3-nitropropionic acid (3-NP), an inhibitor of the mitochondrial citric acid cycle, results in a progressive locomotor deterioration resembling that of HD. Furthermore, we observed congruent with other reports, that 3-NP produces a very selective striatal degeneration. It differs mechanistically from excitotoxic lesions in that 3-NP irreversibly inhibits the mitochondrial citric acid cycle and leads to depressed ATP levels and elevated lactate concentrations. Recent neurochemical studies have implicate...Continue Reading

References

Apr 1, 1992·Neurology·R L Albin, J T Greenamyre
Nov 13, 1986·The New England Journal of Medicine·J B Martin, J F Gusella
Feb 1, 1972·Experimental Neurology·F A Mettler
Nov 17, 1983·Nature·J F GusellaA Y Sakaguchi
Jan 1, 1995·Brain Research Bulletin·C V BorlonganP R Sanberg
Nov 21, 1994·Neuroreport·T K KoutouzisP R Sanberg
Aug 1, 1993·Pharmacology & Therapeutics·C F Zorumski, J W Olney

❮ Previous
Next ❯

Citations

Oct 30, 2008·Archives of Pharmacal Research·Bilgen BasgutIclal Cakici
Mar 27, 2004·European Journal of Pharmacology·Pedro MontillaInmaculada Bujalance
Oct 13, 2006·Antioxidants & Redox Signaling·Mark P Mattson
Mar 19, 2009·ILAR Journal·Christine Gagliardi, Bruce A Bunnell
Feb 18, 2005·NeuroRx : the Journal of the American Society for Experimental NeuroTherapeutics·Stephen B Dunnett, Anne E Rosser
Aug 1, 2002·Proceedings of the National Academy of Sciences of the United States of America·C Dirk KeeneWalter C Low
May 30, 2013·Journal of Ginseng Research·Ik-Hyun Cho
Nov 22, 2011·Behavioural Brain Research·Dagmar H ZeefYasin Temel
Oct 5, 2001·The American Journal of Pathology·K L GabrielsonJ Bressler
Apr 3, 2009·Journal of Molecular and Cellular Cardiology·Sergiy M NadtochiyPaul S Brookes
May 5, 2016·Mechanisms of Ageing and Development·G K ShinomolM M Srinivas Bharath
Jan 9, 2013·Journal of Agricultural and Food Chemistry·David J Smith, Robin C Anderson
Nov 16, 2004·European Journal of Pharmacology·Isaac TúnezRené Drucker-Colín

❮ Previous
Next ❯

Related Concepts

Related Feeds

Antihypertensive Agents: Mechanisms of Action

Antihypertensive drugs are used to treat hypertension (high blood pressure) which aims to prevent the complications of high blood pressure, such as stroke and myocardial infarction. Discover the latest research on antihypertensive drugs and their mechanism of action here.

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.

Attention Disorders

Attention is involved in all cognitive activities, and attention disorders are reported in patients with various neurological diseases. Here are the latest discoveries pertaining to attention disorders.