Lynch-like syndrome: genes and variants
Lynch-like syndrome is linked to 2 analyzed proteins (MLH1 and MSH6). 2 DNA variants are known to cause it; 4 more are uncertain, and 0 of those already look disease-causing on computable evidence.
Last updated 2026-09-30. Research information, not medical advice.
Genes linked to Lynch-like syndrome
MLH1: DNA mismatch repair protein Mlh1
The protein partners with PMS2 to form MutL alpha, a core complex in post-replicative DNA mismatch repair. By helping correct copying errors in DNA, MLH1 protects genome stability, and inherited MLH1 variants are a major cause of Lynch syndrome.
1 disease-causing and 2 uncertain variants in MLH1 are linked to Lynch-like syndrome.
MSH6: DNA mismatch repair protein Msh6
Together with MSH2, it recognizes single-base mismatches and small insertion-deletion loops during DNA replication and initiates mismatch repair. Germline loss-of-function variants cause Lynch syndrome, while biallelic variants can cause constitutional mismatch-repair deficiency.
1 disease-causing and 0 uncertain variants in MSH6 are linked to Lynch-like syndrome.
Weakly linked (only a few uncertain records): MSH2.
Known disease-causing variants in Lynch-like syndrome
| Variant | Position | Protein part | Clinical label |
|---|---|---|---|
| MLH1 S556R | 556 | Interaction with EXO1 | Disease-causing |
| MSH6 H458N | 458 | Disease-causing |
Same protein, different disease
- Lynch syndrome is also caused by MLH1 variants; they fall mostly in different places as the Lynch-like syndrome variants (64 disease-causing).
- Hereditary nonpolyposis colorectal neoplasms is also caused by MLH1 variants; they fall mostly in different places as the Lynch-like syndrome variants (51 disease-causing).
- Colorectal cancer, hereditary nonpolyposis, type 6 is also caused by MLH1 variants; they fall mostly in different places as the Lynch-like syndrome variants (17 disease-causing).
- Muir-Torré syndrome is also caused by MLH1 variants; they fall mostly in different places as the Lynch-like syndrome variants (4 disease-causing).
- Hereditary nonpolyposis colon cancer is also caused by MLH1 variants; they fall mostly in different places as the Lynch-like syndrome variants (4 disease-causing).
- Hereditary nonpolyposis colorectal neoplasms is also caused by MSH6 variants; they fall mostly in different places as the Lynch-like syndrome variants (14 disease-causing).
- Lynch syndrome is also caused by MSH6 variants; they fall mostly in different places as the Lynch-like syndrome variants (12 disease-causing).
- Hereditary nonpolyposis colon cancer is also caused by MSH6 variants; they fall mostly in different places as the Lynch-like syndrome variants (3 disease-causing).
Diseases related to Lynch-like syndrome
- Lynch syndrome, also linked to MLH1 and MSH6
- Hereditary nonpolyposis colorectal neoplasms, also linked to MLH1 and MSH6
- Ovarian cancer, also linked to MLH1 and MSH6
- Colorectal cancer, also linked to MLH1 and MSH6
- Gastric cancer, also linked to MLH1 and MSH6
- Hereditary nonpolyposis colon cancer, also linked to MLH1 and MSH6
- Mismatch repair cancer syndrome, also linked to MLH1 and MSH6
- Breast and/or ovarian cancer, also linked to MLH1 and MSH6
- Endometrial carcinoma, also linked to MLH1 and MSH6
- Colorectal cancer, hereditary nonpolyposis, type 6, also linked to MLH1
- Hereditary breast ovarian cancer syndrome, also linked to MLH1
- Muir-Torré syndrome, also linked to MLH1
Frequently asked questions
Which genes are linked to Lynch-like syndrome?
In CATVariant, Lynch-like syndrome is linked to 2 analyzed proteins: MLH1 (DNA mismatch repair protein Mlh1) and MSH6 (DNA mismatch repair protein Msh6).
How many genetic variants are linked to Lynch-like syndrome?
6 variants: 2 are classified as disease-causing (pathogenic or likely pathogenic) in ClinVar and 4 are of uncertain significance or have conflicting reports.
Which uncertain variants in Lynch-like syndrome look disease-causing?
None of the uncertain variants currently reaches the likely-pathogenic range on computable evidence alone.
About this data
Variant–disease links come from ClinVar, Open Targets and UniProt, pooled from the latest public CATVariant analysis of each human protein. Evidence scores use the ACMG/AMP Bayesian points scale with computable criteria only (position among known disease variants, rarity in gnomAD, calibrated predictors, deep mutational scanning); there is no family or patient data, so they prioritise variants for expert review and never classify them.
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