Animal models Huntington disease

Since the early 1980s, much effort has focused on animal models of acute and chronic neurodegeneration in search of therapeutics for stroke. Neuronal cell death follows strokes, acute ischemic insults, and chronic neurodegeneration, such as Parkinson s disease, Alzheimer s disease (AD), epilepsy, and Huntington s disease. Up to 80% of all strokes result from focal infarcts and ischemia in the middle cerebral artery (MCA), so the commonly used animal models for neuroprotection are produced by temporary or permanent occlusion of the MCA.5 Lesions of the MCA include occlusion by electrocoagulation, intraluminal monofilaments, photochemical effects, thrombosis, and endothelin-1, but all of these models necessitate studying reperfusion events and validating MCA occlusion by behavioral assessments. 

In terms of clinical proof of neuroprotective effects in chronic neurodegenerative diseases, so far there are promising clinical results in ATS only with riluzole, and even then, the increase of survival obtained was only modest. The failure of r emacemide in a recent study in Huntington s disease was clearly a big setback. On the other hand, the moderate-affinity NMDA receptor antagonist memantine provides clear symptomatic improvement in dementia in both clinical and preclinical situations, and the precHnical data predict neuroprotective effects, substantiated by numerous animal models. 

Rubinsztein DC (2002) Lessons from animal models of Huntington s disease. Trends Genet 18 202-209. 

Diguet E, Femagut PO, Wei X, Du Y, Rouland R, Gross C, Bezard E, Tison F (2004) Deleterious effects of minocycline in animal models of Parkinson s disease and Huntington s disease. Eur J Neurosci 19 326 3276. 

Keywords Alzheimer s disease Amyotrophic lateral sclerosis Animal models Apoptosis Huntington s disease Neurodegeneration Neuroprotection Parkinson s disease Randomized controlled trials Therapeutics... 

Anderson, K.D., Panayotatos, N., Corcoran, T.L., Lindsay, R.M., Wiegand, S.J., 1996. Ciliary neurotrophic factor protects striatal output neurons in an animal model of Huntington disease. Proc. Natl. Acad. Sci. U. S. A. 93, 7346-7351. 

There are multitudes of namrally occurring diseases of the central nervous system, including degenerative, traumatic, neoplastic, and metabolic disorders. Naturally occurring diseases for which there are potential models include models for include Parkinson s disease (PD), Huntington s disease (HD), Alzheimer s disease (AD), cerebral ischemia, miscellaneous neoplastic conditions, and lysosomal storage diseases. Examples of selected animal models of human disease will be briefly suimnarized. 

Kimura, H., McGeer, E. G., and McGeer, P. L., 1980, Metabolic alterations in an animal model of Huntington s disease using the C-deoxyglucose method, /. Neural Transm. Suppl. 16 103-109. 

EGCG can also reduce hippocampal neuronal injury induced by transient global ischemia [98]. Neuroprotective effects of flavonoids have also been observed in animal models of Huntington s disease, where the flavonol fisetin has been reported to be effective in reducing pathophysiology through its actions on the extracellular... 

R 415 W.-T. Lee and C. Chang, Magnetic Resonance Imaging and Spectroscopy in Assessing 3-Nitroproprionic Add-Induced Brain Lesions An Animal Model of Huntington s Disease , Prog.NeurobioL, 2004,72,87... 

Matthews, R.T., L. Yang, B.G. Jenkins, R.J. Ferrante, B.R. Rosen, R. Kaddurah-Daouk, and M.F Beal, Neuroprotective effects of creatine and cyclocreatine in animal models of Huntington s disease. J Neurosci, 18 156-163, 1998. 

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