JUL 2014 ARTICLE LIST >>
PharmaTutor (July- 2014)
ISSN: 2347 - 7881
Received On: 09/05/2014; Accepted On: 13/05/2014; Published On: 01/07/2014
AUTHORS: Amandeep Kaur
Department of Pharmacology, G.H.G Khalsa College of Pharmacy
Gurusar Sadhar, Ludhiana, Punjab, INDIA
amananny2011@gmail.com
ABSTRACT:
Huntington's disease is a complex degenerative disorder that affects the central nervous system. Huntington's disease is an autosomal-dominant, progressive neurodegenerative disorder typically presents during mid-life with a distinct phenotype, including chorea and dystonia, incoordination, cognitive decline, and behavioral abnormalities. HD is caused by an expanded CAG repeat in the gene that encodes the protein huntingtin on chromosome 4; this results in progressive atrophy of the striatum as well as cortical and other extra striatal structures. Since 1993, genetic testing has been available to confirm diagnosis in affected adults and for pre-symptomatic testing in at-risk individuals. The review aims majorly on these areas namely: History, etiology, symptoms, pathophysiology, treatment and other aspects of Huntington’s disease.
How to cite this article: A Kaur; Huntington’s Disease: An Update; PharmaTutor; 2014; 2(7); 47-53
[ABSTRACT WITH CITATION] [VIEW AS HTML]
REFERENCES:
1.Roze E1, Bonnet C, Betuing S, Caboche J Huntington's disease. Adv Exp Med Biol 2010;685:45-63.
2.Cowan Maxwell W, Kandal R. E. J Am Med Assoc. 2001:285; 594-600.
3. ninds.nih.gov/disorders/huntington/detail_huntington.htm.
4.Sawa A. Mechanisms for neuronal cell death and dysfunction in Huntington's disease: pathological cross-talk between the nucleus and the mitochondria? J Mol Med (Berl) 2001;79(7):375-81.
5. news-medical.net/health/Huntingtons-Disease-History.aspx#.
6.Harper PS. The epidemiology of Huntington's disease. Hum Genet. 1992;89(4):365-76.
7. umm.edu/Health/Medical/Ency/Articles/Huntingtons-disease.
8. voices.yahoo.com/an-overview-huntingtons-disease-huntington-chorea-7024570.html?cat=70.
9. gponline.com/clinical-review-huntingtons-disease/neurology/article/1090296.
10.Paulsen JS, Ready RE, Hamilton JM, Mega MS,Cummings JL. Neuropsychiatric aspects of Huntington’s disease. J Neurol Neurosurg Psychiatry2001;71:310–14.
11.Van der Burg JM, Bjorkqvist M, Brundin P. Beyond the brain: widespread pathology in Huntington's disease. Lancet Neurol 2009; 8: 765-74.
12.Raymund AC Roos.Huntington’s disease: a clinical review.Orphanet J Rare Dis 2010; 5:40.
13.Hunington's disease collaborative research group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.Cell 1993, 72:971-983.
14.Koliatsos E. V, Schilling Gabrielle, Borchelt, B. David, Becher W. Mark and Ross AC. Mechanisms of Neuronal Death in Huntington’s Disease’. In: Mattson P. Mark, eds. Pathogenesis of Neurodegenerative Disorders, Totowa, New Jercy, Humana press, 2001: 93-111.
15.Cattaneo E. Dysfunction of Wild-Type Huntingtin in Huntington disease. News Physiol Sci 2003; 18:34-37.
16.Browne ES, Ferrante JR, Beal FM. Oxidative Stress in Huntington’s Disease. Brain Pathol 1999;9: 147-163.
17.Gardian G. and Vecsei L. Huntington’s disease: pathomechanism and therapeutic Perspectives. J Neural Transm2004; 111: 1485–1494.
18.Ranum LP, Day JW. Pathogenic RNA repeats: an expanding role in genetic disease. Trends Genet 2004; 20: 506–512.
19.Li LB, Bonini NM. Roles of trinucleotide-repeat RNA in neurological disease and degeneration. Trends Neurosci 2010; 33: 292–298.
20.Martin JB, Gusella JF. Huntington's disease. Pathogenesis and management. New Eng J Med 1986; 315:1267-1276.
21.Zheng G, Dwoskin LP, Crooks PA. Vesicular monoamine transporter 2: role as a novel target for drug development. AAPS J 2006;8(4): 682–692.
22.Barr AN, Fischer JH, Koller WC, Spunt Al, Singhal A. Serum haloperidol concentration and choreiform movements in Huntington's disease. Neurol1988; 38:84-88.
23.White RF, Vasterling JJ, Koroshetz WJ, Myers R. Neuropsychology of Huntington's disease. In: RF White, editor. Clinical syndromes in adult neuropsychology: the practitioners handbook. London: Elsevier Amsterdam, 1992. p. 213-251.
24.Newsom-Davis J, Donaghy M. In: Oxford textbook of medicine. 3rd edition. Weatherall DJ, Ledingham JGG, Warrell DA, editors. Oxford and New York: Oxford University Press; 1996. Neurology; pp. 3815–4136.
25.Folstein SE. Huntington's disease: a disorder of families. Baltimore MD: The Johns Hopkins University Press 1989.
26.Maucksch C1, Vazey EM, Gordon RJ, Connor B. Stem cell-based therapy for Huntington's disease. J Cell Biochem. 2013; 114(4): 754-63.
.png)
