Abstract
Huntington’s disease (HD) is an inheritable autosomal dominant neurodegenerative disorder characterized by a clinical triad of progressive choreiform movements (dance-like movements), psychiatric symptoms, and a decline in the cognitive functions. It is caused by a CAG trinucleotide repeat expansion in the HD gene whereas principal pathology of this disease is the loss of striatal and cortical projection neurons. Different experimental animal models have been figured out to assist in understanding the mechanisms involved in the progress of the HD. Extensive researches had been done for testing a large array of neuroprotective agents using animal models which mimic such disorder and to examine the different mechanisms suggested to contribute in the progress of HD. Excitotoxicity in the central nervous system (CNS) results from glutamate exposure for prolonged periods or to excessive concentrations to glutamate. N-methyl-D-aspartic acid (NMDA) receptor is an ionotropic glutamate receptor responsible for the memory functions and exhibits several features of relevance to neuronal death. Neurotoxicity mediated by NMDA receptor with subsequent loss of striatal neurons contributes in the pathophysiology of HD. The current review focusing on using of NMDA receptor legends as a new strategy in treatment of HD.
Highlights
Neurodegenerative diseases of the basal ganglia such as Parkinson's disease (PD) and Huntington's disease (HD) have received considerable concern, in the past few years
In adult-onset Huntington’s disease (HD), patients show a progressive deterioration of motor and cognitive functions that follows three well-defined stages spread over 15–20 years (Harper, 1991)
Models for HD: A number of animal models of HD have been available for more than 30 years (Brouillet et al, ) in an effort to understand how the mutation causes the symptoms and pathology observed in patients in order to observe the process of cell death in a simpler, more controlled circumstances than the human disorder
Summary
Neurodegenerative diseases of the basal ganglia such as Parkinson's disease (PD) and Huntington's disease (HD) have received considerable concern, in the past few years. Some evidence has outlined that direct interactions between mutant huntingtin and transcription factors may be the proximal defect that leads to transcriptional dysfunction (Tunez et al, 2010) This results in a cascade of compensatory and damaging events as mitochondrial dysfunction, energy depletion, oxidative stress, DNA damage and others that might play a role in neuronal death (Pringsheim et al, 2012). Models for HD: A number of animal models of HD have been available for more than 30 years (Brouillet et al, ) in an effort to understand how the mutation causes the symptoms and pathology observed in patients in order to observe the process of cell death in a simpler, more controlled circumstances than the human disorder
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call