| 1309227 | Mlh3 | mutL homolog 3 | ENCODES a protein that exhibits centromeric DNA binding (ortholog); chromatin binding (ortholog); INVOLVED IN DNA damage response (ortholog); female meiosis I (ortholog); intracellular protein localization (ortholog); PARTICIPATES IN mismatch repair pathway; ASSOCIATED WITH breast cancer (ortholog); Breast Cancer, Familial (ortholog); breast carcinoma (ortholog); FOUND IN chiasma (ortholog); condensed chromosome (ortholog); condensed nuclear chromosome (ortholog); INTERACTS WITH 2,4-dinitrotoluene; 2,6-dinitrotoluene; 4,4'-sulfonyldiphenol | 6 | 110612535 | 110649408 | Rat | 124 | symbol , old_gene_name , PhenoGen | gene, protein-coding, PROVISIONAL [RefSeq] |
| 1319451 | MLH3 | mutL homolog 3 | This gene is a member of the MutL-homolog (MLH) family of DNA mismatch repair (MMR) genes. MLH genes are implicated in maintaining genomic integrity during DNA replication and after meiotic recombination. The protein encoded by this gene functions as a heterodimer with other family members. Somatic mutations in this gene frequently occur in tumors exhibiting microsatellite instability, and germline mutations have been linked to hereditary nonpolyposis colorectal cancer type 7 (HNPCC7). Several alternatively spliced transcript variants have been identified, but the full-length nature of only two transcript variants has been determined. [provided by RefSeq, Jul 2008] | 14 | 75013775 | 75051467 | Human | 547 | symbol , old_gene_name , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 1553165 | Mlh3 | mutL homolog 3 | Enables centromeric DNA binding activity and chromatin binding activity. Acts upstream of or within meiotic nuclear division; mismatch repair; and protein localization. Located in chiasma; male germ cell nucleus; and synaptonemal complex. Part of mismatch repair complex. Is expressed in brain; early conceptus; ileum; reproductive system; and trigeminal nerve. Human ortholog(s) of this gene implicated in Lynch syndrome; colorectal cancer; endometrial cancer; and hereditary nonpolyposis colorectal cancer type 7. Orthologous to human MLH3 (mutL homolog 3). [provided by Alliance of Genome Resources, Jul 2025] | 12 | 85281240 | 85317421 | Mouse | 122 | symbol , old_gene_name , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 8976170 | Mlh3 | mutL homolog 3 | ENCODES a protein that exhibits centromeric DNA binding (ortholog); chromatin binding (ortholog); INVOLVED IN DNA damage response (ortholog); female meiosis I (ortholog); intracellular protein localization (ortholog); PARTICIPATES IN mismatch repair pathway; ASSOCIATED WITH breast cancer (ortholog); Breast Cancer, Familial (ortholog); breast carcinoma (ortholog); FOUND IN chiasma (ortholog); condensed chromosome (ortholog); condensed nuclear chromosome (ortholog) | NW_004955523 | 515409 | 547257 | Chinchilla | 50 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 11831005 | MLH3 | mutL homolog 3 | ENCODES a protein that exhibits ATP binding (inferred); ATP hydrolysis activity (inferred); ATP-dependent DNA damage sensor activity (inferred); INVOLVED IN DNA damage response (ortholog); female meiosis I (ortholog); intracellular protein localization (ortholog); PARTICIPATES IN mismatch repair pat hway; ASSOCIATED WITH breast cancer (ortholog); Breast Cancer, Familial (ortholog); breast carcinoma (ortholog); FOUND IN chiasma (inferred); condensed chromosome (inferred); condensed nuclear chromosome (inferred) | 14 | 55568033 | 55603672 | Bonobo | 59 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 12076576 | MLH3 | mutL homolog 3 | ENCODES a protein that exhibits ATP binding (inferred); ATP hydrolysis activity (inferred); ATP-dependent DNA damage sensor activity (inferred); INVOLVED IN DNA damage response (ortholog); female meiosis I (ortholog); intracellular protein localization (ortholog); PARTICIPATES IN mismatch repair pat hway; ASSOCIATED WITH breast cancer (ortholog); Breast Cancer, Familial (ortholog); breast carcinoma (ortholog); FOUND IN chiasma (inferred); condensed chromosome (inferred); condensed nuclear chromosome (inferred) | 8 | 48123263 | 48153989 | Dog | 59 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 12560438 | Mlh3 | mutL homolog 3 | ENCODES a protein that exhibits ATP binding (inferred); ATP hydrolysis activity (inferred); ATP-dependent DNA damage sensor activity (inferred); INVOLVED IN DNA damage response (ortholog); female meiosis I (ortholog); intracellular protein localization (ortholog); PARTICIPATES IN mismatch repair pat hway; ASSOCIATED WITH breast cancer (ortholog); Breast Cancer, Familial (ortholog); breast carcinoma (ortholog); FOUND IN chiasma (inferred); condensed chromosome (inferred); condensed nuclear chromosome (inferred) | NW_004936488 | 4164322 | 4195408 | Squirrel | 59 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 20866838 | MLH3 | mutL homolog 3 | | | | | Green Monkey | | symbol | gene, protein-coding |
| 14380642 | MLH3 | | | | | | Yeast | | symbol | gene, null |
| 18776862 | MLH3 | mutL homolog 3 | ENCODES a protein that exhibits ATP binding (inferred); ATP hydrolysis activity (inferred); ATP-dependent DNA damage sensor activity (inferred); INVOLVED IN DNA damage response (ortholog); female meiosis I (ortholog); intracellular protein localization (ortholog); PARTICIPATES IN mismatch repair pat hway; ASSOCIATED WITH breast cancer (ortholog); Breast Cancer, Familial (ortholog); breast carcinoma (ortholog); FOUND IN chiasma (inferred); condensed chromosome (inferred); condensed nuclear chromosome (inferred) | | | | Green Monkey | 59 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 18901041 | Mlh3 | mutL homolog 3 | ENCODES a protein that exhibits ATP binding (inferred); ATP hydrolysis activity (inferred); ATP-dependent DNA damage sensor activity (inferred); INVOLVED IN DNA damage response (ortholog); female meiosis I (ortholog); intracellular protein localization (ortholog); PARTICIPATES IN mismatch repair pat hway; ASSOCIATED WITH breast cancer (ortholog); Breast Cancer, Familial (ortholog); breast carcinoma (ortholog); FOUND IN chiasma (inferred); condensed chromosome (inferred); condensed nuclear chromosome (inferred) | | | | Naked Mole-Rat | 59 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 155247094 | mlh3 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 625964784 | Mlh3 | mutL homolog 3 | ENCODES a protein that exhibits centromeric DNA binding (ortholog); chromatin binding (ortholog); INVOLVED IN DNA damage response (ortholog); female meiosis I (ortholog); intracellular protein localization (ortholog); PARTICIPATES IN mismatch repair pathway; ASSOCIATED WITH breast cancer (ortholog); Breast Cancer, Familial (ortholog); breast carcinoma (ortholog); FOUND IN chiasma (ortholog); condensed chromosome (ortholog); condensed nuclear chromosome (ortholog) | | | | Black Rat | 43 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 155240599 | mlh3.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 12624126 | LOC101972693 | DNA mismatch repair protein Mlh3-like | | NW_004936582 | 3173186 | 3175806 | Squirrel | | name | gene, pseudo, MODEL [RefSeq] |
| 13993043 | ACYP1 | acylphosphatase 1 | ENCODES a protein that exhibits acylphosphatase activity (inferred); ATP binding (inferred); ATP hydrolysis activity (inferred); INVOLVED IN DNA damage response (inferred); mismatch repair (inferred); PARTICIPATES IN Leigh disease pathway; primary hyperoxaluria type 2 pathway; pyruvate decarboxylase deficiency pathway; FOUND IN chiasma (inferred); condensed chromosome (inferred); condensed nuclear chromosome (inferred) | | | | Pig | 30 | ensembl_gene_symbol | gene, protein-coding, VALIDATED [RefSeq] |
| 733729 | MLH1 | mutL homolog 1 | The protein encoded by this gene can heterodimerize with mismatch repair endonuclease PMS2 to form MutL alpha, part of the DNA mismatch repair system. When MutL alpha is bound by MutS beta and some accessory proteins, the PMS2 subunit of MutL alpha introduces a single-strand break near DNA mismatche s, providing an entry point for exonuclease degradation. The encoded protein is also involved in DNA damage signaling and can heterodimerize with DNA mismatch repair protein MLH3 to form MutL gamma, which is involved in meiosis. This gene was identified as a locus frequently mutated in hereditary nonpolyposis colon cancer (HNPCC). [provided by RefSeq, Aug 2017] | 3 | 36993466 | 37050846 | Human | 4553 | description | gene, protein-coding, REVIEWED [RefSeq] |
