Tumour characteristics provide evidence for germline mismatch repair missense variant pathogenicity

S Li, D Qian, BA Thompson, S Gutierrez… - Journal of medical …, 2020 - jmg.bmj.com
Background Pathogenic variants in mismatch repair (MMR) genes (MLH1, MSH2, MSH6
and PMS2) increase risk for Lynch syndrome and related cancers. We quantified tumour …

Germline mismatch repair gene variants analyzed by universal sequencing in Japanese cancer patients

Y Kiyozumi, H Matsubayashi, Y Horiuchi… - Cancer …, 2019 - Wiley Online Library
Background Lynch syndrome (LS) is the commonest inherited cancer syndrome caused by
pathogenic variants of germline DNA mismatch repair (g. MMR) genes. Genome‐wide …

Determining the frequency of de novo germline mutations in DNA mismatch repair genes

AK Win, MA Jenkins, DD Buchanan… - Journal of medical …, 2011 - jmg.bmj.com
Background Carriers of a germline mutation in a DNA mismatch repair (MMR) gene—that is,
persons with Lynch syndrome—have substantially high risks of colorectal (CRC) …

Constitutional mismatch repair deficiency mimicking Lynch syndrome is associated with hypomorphic mismatch repair gene variants

R Gallon, C Brekelmans, M Martin, V Bours… - npj Precision …, 2024 - nature.com
Lynch syndrome (LS) and constitutional mismatch repair deficiency (CMMRD) are distinct
cancer syndromes caused, respectively, by mono-and bi-allelic germline mismatch repair …

[HTML][HTML] Germline and tumor sequencing as a diagnostic tool to resolve suspected Lynch syndrome

BJ Pope, M Clendenning, C Rosty, K Mahmood… - The Journal of Molecular …, 2021 - Elsevier
Patients in whom mismatch repair (MMR)-deficient cancer develops in the absence of
pathogenic variants of germline MMR genes or somatic hypermethylation of the MLH1 gene …

A functional assay–based procedure to classify mismatch repair gene variants in Lynch syndrome

M Drost, Y Tiersma, BA Thompson… - Genetics in …, 2019 - nature.com
Purpose To enhance classification of variants of uncertain significance (VUS) in the DNA
mismatch repair (MMR) genes in the cancer predisposition Lynch syndrome, we developed …

Gene-specific features (MLH1, MSH2, MSH6, PMS2) of mismatch repair (MMR) protein expression and somatic mutations (muts), microsatellite instability (MSI) and …

MJ Hall, JN Bodor, J Xiu, R Feldman, A Grothey… - 2019 - ascopubs.org
505 Background: MSI is a tumor biomarker for immunotherapy efficacy that is closely
associated w/deficient MMR, and is also variably associated w/somatic muts of the MMR …

Early age of onset and broad cancer spectrum persist in MSH6-and PMS2-associated Lynch syndrome

YL Liu, KA Cadoo, A Maio, Z Patel, Y Kemel… - Genetics in …, 2022 - Elsevier
Purpose This study aimed to characterize MSH6/PMS2-associated mismatch repair–
deficient (MMR-D)/microsatellite instability-high (MSI-H) tumors, given revised guidelines …

[HTML][HTML] Microsatellite instability is associated with the presence of Lynch syndrome pan-cancer

A Latham, P Srinivasan, Y Kemel, J Shia… - Journal of clinical …, 2019 - ncbi.nlm.nih.gov
PURPOSE Microsatellite instability (MSI) and/or mismatch repair deficiency (MMR-D) testing
has traditionally been performed in patients with colorectal (CRC) and endometrial cancer …

Cancer risks by gene, age, and gender in 6350 carriers of pathogenic mismatch repair variants: findings from the Prospective Lynch Syndrome Database

M Dominguez-Valentin, JR Sampson, TT Seppälä… - Genetics in …, 2020 - nature.com
Purpose Pathogenic variants affecting MLH1, MSH2, MSH6, and PMS2 cause Lynch
syndrome and result in different but imprecisely known cancer risks. This study aimed to …