Human MutLγ, the MLH1–MLH3 heterodimer, is an endonuclease that promotes DNA expansion

CAG Repeat Not Polyglutamine Length Determines Timing of Huntington’s Disease Onset

(2019) Jong-Min Lee et al.   CELL

A genetic association study of glutamine-encoding DNA sequence structures, somatic CAG expansion, and DNA repair gene variants, with Huntington disease clinical outcomes

(2019) Marc Ciosi et al.   EBioMedicine

GAA•TTC repeat expansion in human cells is mediated by mismatch repair complex MutLγ and depends upon the endonuclease domain in MLH3 isoform one

(2018) Anasheh Halabi et al.   NUCLEIC ACIDS RESEARCH

Mechanisms of genetic instability caused by (CGG)n repeats in an experimental mammalian system

(2018) Artem V. Kononenko et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

MutLγ promotes repeat expansion in a Fragile X mouse model while EXO1 is protective

(2018) Xiaonan Zhao et al.   PLoS Genetics

A modifier of Huntington's disease onset at the MLH1 locus

(2017) Jong-Min Lee et al.   HUMAN MOLECULAR GENETICS

Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study

(2017) Davina J Hensman Moss et al.   LANCET NEUROLOGY

The mismatch repair and meiotic recombination endonuclease Mlh1-Mlh3 is activated by polymer formation and can cleave DNA substrates in trans

(2017) Carol M. Manhart et al.   PLOS BIOLOGY

DNA repair pathways underlie a common genetic mechanism modulating onset in polyglutamine diseases

(2016) Conceição Bettencourt et al.   ANNALS OF NEUROLOGY

Endonuclease activities of MutLα and its homologs in DNA mismatch repair

(2016) Lyudmila Y. Kadyrova et al.   DNA REPAIR

Roles for mismatch repair family proteins in promoting meiotic crossing over

(2016) Carol M. Manhart et al.   DNA REPAIR

Disease-associated repeat instability and mismatch repair

(2016) Monika H.M. Schmidt et al.   DNA REPAIR

The Major Replicative Histone Chaperone CAF-1 Suppresses the Activity of the DNA Mismatch Repair System in the Cytotoxic Response to a DNA-methylating Agent

(2016) Lyudmila Y. Kadyrova et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Crosstalk between MSH2–MSH3 and polβ promotes trinucleotide repeat expansion during base excision repair

(2016) Yanhao Lai et al.   Nature Communications

A MutSβ-Dependent Contribution of MutSα to Repeat Expansions in Fragile X Premutation Mice?

(2016) Xiao-Nan Zhao et al.   PLoS Genetics

DNA Triplet Repeat Expansion and Mismatch Repair

(2015) Ravi R. Iyer et al.   Annual Review of Biochemistry

Eukaryotic Mismatch Repair in Relation to DNA Replication

(2015) Thomas A. Kunkel et al.   Annual Review of Genetics

Identification of Genetic Factors that Modify Clinical Onset of Huntington’s Disease

(2015) Jong-Min Lee et al.   CELL

Mutsβ generates both expansions and contractions in a mouse model of the Fragile X-associated disorders

(2015) Xiao-Nan Zhao et al.   HUMAN MOLECULAR GENETICS

Heterozygosity for a Hypomorphic Polβ Mutation Reduces the Expansion Frequency in a Mouse Model of the Fragile X-Related Disorders

(2015) Rachel Adihe Lokanga et al.   PLoS Genetics

Mlh1-Mlh3, a Meiotic Crossover and DNA Mismatch Repair Factor, Is a Msh2-Msh3-stimulated Endonuclease

(2014) Maria V. Rogacheva et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TheSaccharomyces cerevisiaeMlh1-Mlh3 Heterodimer Is an Endonuclease That Preferentially Binds to Holliday Junctions

(2014) Lepakshi Ranjha et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Modelling and inference reveal nonlinear length-dependent suppression of somatic instability for small disease associated alleles in myotonic dystrophy type 1 and Huntington disease

(2013) C. F. Higham et al.   Journal of the Royal Society Interface

Structure of the MutLα C-terminal domain reveals how Mlh1 contributes to Pms1 endonuclease site

(2013) Emeric Gueneau et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Extrahelical (CAG)/(CTG) triplet repeat elements support proliferating cell nuclear antigen loading and MutL  endonuclease activation

(2013) A. Pluciennik et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Detection of Slipped-DNAs at the Trinucleotide Repeats of the Myotonic Dystrophy Type I Disease Locus in Patient Tissues

(2013) Michelle M. Axford et al.   PLoS Genetics

Mismatch Repair Genes Mlh1 and Mlh3 Modify CAG Instability in Huntington's Disease Mice: Genome-Wide and Candidate Approaches

(2013) Ricardo Mouro Pinto et al.   PLoS Genetics

CAF-I-dependent control of degradation of the discontinuous strands during mismatch repair

(2011) L. Y. Kadyrova et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

MutLα and Proliferating Cell Nuclear Antigen Share Binding Sites on MutSβ

(2010) Ravi R. Iyer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Repeat expansion disease: progress and puzzles in disease pathogenesis

(2010) Albert R. La Spada et al.   NATURE REVIEWS GENETICS

Repeat instability as the basis for human diseases and as a potential target for therapy

(2010) Arturo López Castel et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

PCNA function in the activation and strand direction of MutL  endonuclease in mismatch repair

(2010) A. Pluciennik et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A possible mechanism for exonuclease 1-independent eukaryotic mismatch repair

(2009) F. A. Kadyrov et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The PMS2 Subunit of Human MutLα Contains a Metal Ion Binding Domain of the Iron-Dependent Repressor Protein Family

(2008) Jan Kosinski et al.   JOURNAL OF MOLECULAR BIOLOGY

DNA instability in postmitotic neurons

(2008) R. Gonitel et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA