Impairment of Cellular Transcriptional Machinery in Poly(Q) Disorders

Polyglutamine or poly(Q) diseases represent a group of fatal human disorders which exhibit some common neurodegenerative symptoms and share somewhat similar mechanism of pathogenesis. Some of the common clinical symptoms of poly(Q) diseases include progressive loss of body coordination, memory, difficulty in speech and intellectual disabilities [1]. Most forms of the poly(Q) disorders are dominantly inherited, exhibit age dependent phenotypic manifestations, progressive in nature and result in degeneration of specific group of neurons in the brain as per the characteristics of each disease type [2,3]. Some of the commonly known poly(Q) disorders include Spinal and Bulbar Muscular Atrophy (SBMA), Huntington’s Disease (HD), six of the Spinocerebellar ataxias (SCA1, 2, 3, 6, 7 and 17) and Dentato Rubral Pallidoluysian Atrophy (DRPLA) [2,4].