Huntington's and myotonic dystrophy hESCs: down-regulated trinucleotide repeat instability and mismatch repair machinery expression upon differentiation

Evidence that hMLH3 functions primarily in meiosis and in hMSH2-hMSH3 mismatch repair

(2010) Nicole Charbonneau et al.   CANCER BIOLOGY & THERAPY

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

Tissue- and age-specific DNA replication patterns at the CTG/CAG-expanded human myotonic dystrophy type 1 locus

(2010) John D Cleary et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Isolated short CTG/CAG DNA slip-outs are repaired efficiently by hMutSβ, but clustered slip-outs are poorly repaired

(2010) Gagan B. Panigrahi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Efficient differentiation of human embryonic stem cells into a homogeneous population of osteoprogenitor-like cells

(2010) Ileana Mateizel et al.   REPRODUCTIVE BIOMEDICINE ONLINE

Human embryonic stem cell models of Huntington disease

(2010) JC Niclis et al.   REPRODUCTIVE BIOMEDICINE ONLINE

Diverse effects of individual mismatch repair components on transcription-induced CAG repeat instability in human cells

(2009) Yunfu Lin et al.   DNA REPAIR

CTG/CAG Repeat Instability Is Modulated by the Levels of Human DNA Ligase I and Its Interaction with Proliferating Cell Nuclear Antigen

(2009) Arturo López Castel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

MSH2 ATPase Domain Mutation Affects CTG•CAG Repeat Instability in Transgenic Mice

(2009) Stéphanie Tomé et al.   PLoS Genetics

Stoichiometry of Base Excision Repair Proteins Correlates with Increased Somatic CAG Instability in Striatum over Cerebellum in Huntington's Disease Transgenic Mice

(2009) Agathi-Vassiliki Goula et al.   PLoS Genetics

Mutagenic roles of DNA “repair” proteins in antibody diversity and disease-associated trinucleotide repeat instability

(2008) Meghan M. Slean et al.   DNA REPAIR

CTG repeat instability in a human embryonic stem cell line carrying the myotonic dystrophy type 1 mutation

(2008) N. De Temmerman et al.   MOLECULAR HUMAN REPRODUCTION

Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes

(2008) Ella Dragileva et al.   NEUROBIOLOGY OF DISEASE

Striatal progenitors derived from human ES cells mature into DARPP32 neurons in vitro and in quinolinic acid-lesioned rats

(2008) L. Aubry et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA