HUNTINGTON'S DISEASE: UNDERSTANDING THE PATHOPHYSIOLOGY THROUGH THE HUNTINGTIN GENE

Huntington's Disease (HD) is a progressive neurodegenerative disorder. It is an autosomal dominant disorder that is categorized by motor dysfunctions, behavioral and cognitive deficits. Reason for this disease is expansion of the polyglutamine (due to the more CAG repeat) in the amino-terminal region of the exon 1 of the Huntingtin gene (HTT). Furthermore, the mutant HTT gene is occupied in the HD associated changes of neurotransmission for enabling the neurodegeneration. Even though the the important pathophysiology of the HD happens in the caudate and putamen, rest regions of the brain are similarly influenced and also show a significant characteristic in the HD pathophysiology. Until now actual remedy for the HD is not available. As a result, current approaches are directing to the HTT gene expression silencing. It is now taken as the probable way of the management of HD. But the most important thing is, core functions of the HTT gene in the brain of adult subject are presently not clear at all and henceforward the outcome of the continued HTT gene expression suppression of is unpredictable. It could be possibly being tough. This review is based on the pathophysiology of HTT on HD.