Cargando…
All three mammalian MutL complexes are required for repeat expansion in a mouse cell model of the Fragile X-related disorders
Expansion of a CGG-repeat tract in the 5’ untranslated region of the FMR1 gene causes the fragile X-related disorders (FXDs; aka the FMR1 disorders). The expansion mechanism is likely shared by the 35+ other diseases resulting from expansion of a disease-specific microsatellite, but many steps in th...
Autores principales: | Miller, Carson J., Kim, Geum-Yi, Zhao, Xiaonan, Usdin, Karen |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2020
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7347238/ https://www.ncbi.nlm.nih.gov/pubmed/32589669 http://dx.doi.org/10.1371/journal.pgen.1008902 |
Ejemplares similares
-
MutLγ promotes repeat expansion in a Fragile X mouse model while EXO1 is protective
por: Zhao, Xiaonan, et al.
Publicado: (2018) -
A MutSβ-Dependent Contribution of MutSα to Repeat Expansions in Fragile X Premutation Mice?
por: Zhao, Xiao-Nan, et al.
Publicado: (2016) -
MutS/MutL crystal structure reveals that the MutS sliding clamp loads MutL onto DNA
por: Groothuizen, Flora S, et al.
Publicado: (2015) -
CGG Repeat Expansion, and Elevated Fmr1 Transcription and Mitochondrial Copy Number in a New Fragile X PM Mouse Embryonic Stem Cell Model
por: Gazy, Inbal, et al.
Publicado: (2020) -
Modifiers of Somatic Repeat Instability in Mouse Models of Friedreich Ataxia and the Fragile X-Related Disorders: Implications for the Mechanism of Somatic Expansion in Huntington’s Disease
por: Zhao, Xiaonan, et al.
Publicado: (2021)