Partners in crime: Proteins implicated in RNA repeat expansion diseases.

ABSTRACT: Short tandem repeats are repetitive nucleotide sequences robustly distributed in the human genome. Their expansion underlies the pathogenesis of multiple neurological disorders, including Huntington's disease, amyotrophic lateral sclerosis, and frontotemporal dementia, fragile X-associated tremor/ataxia syndrome, and myotonic dystrophies, known as repeat expansion disorders (REDs). Several molecular pathomechanisms associated with toxic RNA containing expanded repeats (RNA^exp ) are shared among REDs and contribute to disease progression, however, detailed mechanistic insight into those processes is limited. To deepen our understanding of the interplay between toxic RNA^exp molecules and multiple protein partners, in this review, we discuss the roles of selected RNA-bin

SUBMITTER: Baud A 

PROVIDER: S-EPMC9539487 | biostudies-literature | 2022 Jul

REPOSITORIES: biostudies-literature