Atlas One · Host Biology · Scale 03 — Molecular

MLH1

MutL homolog 1; forms MutLα heterodimer with PMS2 → mismatch repair (MMR) of replication errors. MLH1 loss → MSI-H phenotype and TMB-high tumors responsive to PD-1 blockade. Germline MLH1 mutations cause Lynch syndrome (colorectal, endometrial, ovarian, gastric cancers).

Also known as: MLH1, MutL homolog 1, MutLα, mismatch repair, MMR, Lynch syndrome, MSI-H, dMMR, HNPCC, microsatellite instability

Scale 03 — MolecularScale
draftStatus
2026-06-06Last Reviewed
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mlh1

MLH1

MutL homolog 1; forms MutLα heterodimer with PMS2 → mismatch repair (MMR) of replication errors. MLH1 loss → MSI-H phenotype and TMB-high tumors responsive to PD-1 blockade. Germline MLH1 mutations cause Lynch syndrome (colorectal, endometrial, ovarian, gastric cancers).

Entry Metadata

FieldValue
IDmlh1
NameMLH1
Statusdraft
Last reviewed2026-06-06
Atlas01-human
Scale03-molecular

Cross-Atlas Connections

connects-toP53 connects-toPd 1 connects-toWnt Beta Catenin connects-toTgf Beta connects-toLynch Syndrome

Sources