A plant-based mutant huntingtin model-driven discovery of impaired expression of GTPCH and DHFR.

ABSTRACT: Pathophysiology associated with Huntington's disease (HD) has been studied extensively in various cell and animal models since the 1993 discovery of the mutant huntingtin (mHtt) with abnormally expanded polyglutamine (polyQ) tracts as the causative factor. However, the sequence of early pathophysiological events leading to HD still remains elusive. To gain new insights into the early polyQ-induced pathogenic events, we expressed Htt exon1 (Htt_ex1) with a normal (21), or an extended (42 or 63) number of polyQ in tobacco plants. Here, we show that transgenic plants accumulated Htt_ex1 proteins with corresponding polyQ tracts, and mHtt_ex1 induced protein aggregation and affected plant growth, especially root and root hair development, in a polyQ length-dependen

SUBMITTER: Hung CY 

PROVIDER: S-EPMC9576654 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature