J Steroid Biochem Mol Biol 2020 03 9;197:105526. Epub 2019 Nov 9.
School of Life Sciences, Bharathidasan University, Tiruchirappalli 620024, Tamil Nadu, India; Laboratory of Stem Cells and Neuroregeneration, Department of Animal Science, School of Life Sciences, Bharathidasan University, Tiruchirappalli 620024, Tamil Nadu, India; Faculty Recharge Programme, University Grants Commission (UGC-FRP), New Delhi, India. Electronic address:
Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative disorder mainly affecting the structure and functions of the striatum, cerebral cortex and hippocampus leading to movement disorders, cognitive dysfunctions and emotional disturbances. The onset of HD has been linked to a pathogenic CAG repeat expansion in the huntingtin (HTT) gene that encodes for the polyglutamine (polyQ) stretches in the huntingtin (Htt) protein. Notably, the neuropathogenic events of the mutant HTT gene appear to be primed during adulthood and magnified along the ageing process. Read More