Article

FAN1 nuclease processes and pauses on disease-associated slipped-DNA repeats: Mechanism against repeat expansions

2021-04-16

Abstract excerpt

<h4>Summary</h4> FAN1 nuclease is a modifier of repeat expansion diseases, including Huntington’s disease (HD), fragile X syndrome, and autism. The age of HD onset correlates with ongoing ‘inchworm-like’ repeat expansions (1-3 CAG units/event) in HD brains, and is regulated by three modifiers: The first two, repeat tract length and purity exert their effects by enhancing and slowing CAG expansions, respectively,...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 55a5f3cb-dc81-5bb2-a9f3-dbc0ccaf5c3c
DOI: 10.1101/2021.04.15.439995

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

FAN1 nuclease processes and pauses on disease-associated slipped-DNA repeats: Mechanism against repeat expansionsDOI 10.1101/2021.04.15.439995Read

FAN1 exo- not endo-nuclease pausing on disease-associated slipped-DNA repeats: A mechanism of repeat instability.Semantic proximity Read

FAN1 nuclease activity affects CAG expansion and age at onset of Huntington’s diseaseSemantic proximity Read

DNA extrusion size determines pathway choice during CAG repeat expansionSemantic proximity Read

Structural and molecular basis of FAN1 defects in promoting Huntington's diseaseSemantic proximity Read

DCLRE1A orchestrates CAG repeat contraction following Cas12a-induced DNA breaksSemantic proximity Read

Structural and molecular basis of FAN1 defects in promoting Huntington’s diseaseSemantic proximity Read

A slipped-CAG DNA-binding small molecule induces trinucleotide-repeat contractions in vivo.Semantic proximity Read

Pathogenic CANVAS (AAGGG) <sub>n</sub> repeats stall DNA replication due to the formation of alternative DNA structuresSemantic proximity Read

Increased FAN1 expression by mRNA-LNP attenuates CAG repeat expansion in Huntington patients’ iPSC-derived astrocytesSemantic proximity Read

Unscheduled DNA synthesis reveals a DNA repair hotspot and biomarker of somatic instability at the expanded-CAG repeat tract in the huntingtin geneSemantic proximity Read

Antagonistic roles of canonical and alternative RPA in tandem CAG repeat diseasesSemantic proximity Read

Genetic and Functional Analyses Point to FAN1 as the Source of Multiple Huntington Disease Modifier Effects.Semantic proximity Read