Targeted MS validation of huntingtin protein interaction signatures in Huntington's disease mouse models

Huntington’s disease (HD) is a debilitating progressive neurodegenerative disorder that has profound effects on an individual’s cognitive, motor, and behavioral functions. HD is caused by mutation in the huntingtin (HTT) gene that results in CAG repeat expansion and production of an aggregation-prone polyglutamine-containing protein (mHTT). The expression of mHTT causes selective degeneration, mainly in the striatum and cortex brain regions. Yet, the molecular signatures that underlie their selective sensitization remain incompletely characterized.