Marie Curie Career Integration Grants (CIG)
Protein aggregation is a typical trait of a number of neurodegenerative diseases associated with aging. The genes responsible for modulating the aggregation are not completely known. There is an inverse correlation between the number of the CAG triplets within the huntingtin gene, and the age of onset of symptoms in patients of Huntington's disease (HD). However, there is a wide variation in age of onset of the disease, among carriers of short but toxic CAG tandems, which suggests that the genetic background of the patients strongly influences the severity of the disease. Therefore, the objective of this proposal is to generate new in vivo models of HD to find molecules that modulate protein and RNA toxicity and disease progression.
We are generating in vivo models of HD in nematodes which will be used to find disease modulating genes. We are focusing not only in finding genes involved in protein toxicity dynamics, but also in genes that are involved in the toxicity produced by the nascent RNA containing expanded CAG triplets.
Mechanisms of cell dysfunction by aggregation dynamics of polyQ-containing proteins
PI: Rafael Vázquez-Manrique
Technician: Loli Sequedo