Research progress in molecular pathology of Huntington's disease / 中国药理学通报

ABSTRACT

Huntington's disease is a fetal neurological disorder manifested as movement disorder accompanied by cognitive and psychological impairments. The disease is inherited as autosomal dominant. Huntington's disease is caused by an expansion of a polyglutamine tract in a protein named huntingtin. The length of polyglutamine tract in huntingtin in normal individual is less than 35 glutamines. In Huntington's disease patients the length of polyglutamine tract increases to more than 37 glutamines. The pathogenic mechanisms by which mutant huntingtin causes Huntington's disease have not been fully understood. This paper reviews main progresses in studying the pathogenic mechanisms of mutant huntingtin.