Measuring Repeat-Associated Non-AUG (RAN) Translation.
Sign Up to like & getrecommendations! Published in 2022 at "Methods in molecular biology"
DOI: 10.1007/978-1-0716-1975-9_8
Abstract: Expansions of short nucleotide repeats account for more than 50 neurological or neuromuscular diseases. Many repeat expansion-containing RNAs can generate toxic repeat proteins through repeat-associated non-AUG (RAN) translation in all the reading frames. Understanding how… read more here.
Keywords: repeat associated; associated non; ran translation; non aug ... See more keywords
SCA31 Flies Perform in a Balancing Act between RAN Translation and RNA-Binding Proteins
Sign Up to like & getrecommendations! Published in 2017 at "Neuron"
DOI: 10.1016/j.neuron.2017.03.030
Abstract: In this issue of Neuron, Ishiguro et al. (2017) explore the toxicity of RAN translation in spinocerebellar ataxia 31. Using a Drosophila model, the authors demonstrate that TDP-43 and other RNA-binding proteins act as chaperones to… read more here.
Keywords: binding proteins; rna; rna binding; ran translation ... See more keywords
RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats
Sign Up to like & getrecommendations! Published in 2019 at "Nature neuroscience"
DOI: 10.1038/s41593-019-0455-7
Abstract: Nucleotide repeat expansions in the C9orf72 gene are the most common cause of amyotrophic lateral sclerosis and frontotemporal dementia. Unconventional translation (RAN translation) of C9orf72 repeats generates dipeptide repeat proteins that can cause neurodegeneration. We… read more here.
Keywords: translation c9orf72; rps25 required; nucleotide repeat; ran translation ... See more keywords
Mechanisms of repeat-associated non-AUG translation in neurological microsatellite expansion disorders
Sign Up to like & getrecommendations! Published in 2021 at "Biochemical Society Transactions"
DOI: 10.1042/bst20200690
Abstract: Repeat-associated non-AUG (RAN) translation was discovered in 2011 in spinocerebellar ataxia type 8 (SCA8) and myotonic dystrophy type 1 (DM1). This non-canonical form of translation occurs in all reading frames from both coding and non-coding… read more here.
Keywords: translation; repeat; expansion disorders; repeat associated ... See more keywords
Mechanistic convergence across initiation sites for RAN translation in fragile X associated tremor ataxia syndrome.
Sign Up to like & getrecommendations! Published in 2022 at "Human molecular genetics"
DOI: 10.1093/hmg/ddab353
Abstract: Repeat associated non-AUG (RAN) translation of CGG repeats in the 5'UTR of FMR1 produces toxic proteins that contribute to Fragile X-associated tremor/ataxia syndrome (FXTAS) pathogenesis. The most abundant RAN product, FMRpolyG, initiates predominantly at an… read more here.
Keywords: ran translation; tremor ataxia; fragile associated; associated tremor ... See more keywords