Ontology highlight
ABSTRACT:
SUBMITTER: Figueiredo F
PROVIDER: S-EPMC9222203 | biostudies-literature | 2022 Jun
REPOSITORIES: biostudies-literature
Figueiredo Francisco F Lopes-Marques Mónica M Almeida Bruno B Matscheko Nena N Martins Pedro M PM Silva Alexandra A Macedo-Ribeiro Sandra S
Cells 20220619 12
Spinocerebellar ataxia type 3 (SCA3) is caused by the expansion of a glutamine repeat in the protein ataxin-3, which is deposited as intracellular aggregates in affected brain regions. Despite the controversial role of ataxin-3 amyloid structures in SCA3 pathology, the identification of molecules with the capacity to prevent aberrant self-assembly and stabilize functional conformation(s) of ataxin-3 is a key to the development of therapeutic solutions. Amyloid-specific kinetic assays are routine ...[more]