MLH1
UniProt ID: P40692
Entrez ID: 4292
Protein name: DNA mismatch repair protein Mlh1 (MutL protein homolog 1)
External links: UniprotKB HGNC RefSeq COSMIC
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FUNCTION: Heterodimerizes with PMS2 to form MutL alpha, a component of the post-replicative DNA mismatch repair system (MMR). DNA repair is initiated by MutS alpha (MSH2-MSH6) or MutS beta (MSH2-MSH3) binding to a dsDNA mismatch, then MutL alpha is recruited to the heteroduplex. Assembly of the MutL...
FUNCTION: Heterodimerizes with PMS2 to form MutL alpha, a component of the post-replicative DNA mismatch repair system (MMR). DNA repair is initiated by MutS alpha (MSH2-MSH6) or MutS beta (MSH2-MSH3) binding to a dsDNA mismatch, then MutL alpha is recruited to the heteroduplex. Assembly of the MutL-MutS-heteroduplex ternary complex in presence of RFC and PCNA is sufficient to activate endonuclease activity of PMS2. It introduces single-strand breaks near the mismatch and thus generates new entry points for the exonuclease EXO1 to degrade the strand containing the mismatch. DNA methylation would prevent cleavage and therefore assure that only the newly mutated DNA strand is going to be corrected. MutL alpha (MLH1-PMS2) interacts physically with the clamp loader subunits of DNA polymerase III, suggesting that it may play a role to recruit the DNA polymerase III to the site of the MMR. Also implicated in DNA damage signaling, a process which induces cell cycle arrest and can lead to apoptosis in case of major DNA damages. Heterodimerizes with MLH3 to form MutL gamma which plays a role in meiosis. {ECO:0000269|PubMed:16873062, ECO:0000269|PubMed:18206974, ECO:0000269|PubMed:20020535, ECO:0000269|PubMed:21120944, ECO:0000269|PubMed:9311737}.
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GO - Biological processes (BP):
double-strand break repair via nonhomologous end joining [GO:0006303]; female meiosis chromosome segregation [GO:0016321]; homologous chromosome pairing at meiosis [GO:0007129]; intrinsic apoptotic signaling pathway in response to DNA damage [GO:0008630]; isotype switching [GO:0045190]; male meiosis...
double-strand break repair via nonhomologous end joining [GO:0006303]; female meiosis chromosome segregation [GO:0016321]; homologous chromosome pairing at meiosis [GO:0007129]; intrinsic apoptotic signaling pathway in response to DNA damage [GO:0008630]; isotype switching [GO:0045190]; male meiosis chromosome segregation [GO:0007060]; meiotic metaphase I plate congression [GO:0043060]; meiotic spindle midzone assembly [GO:0051257]; meiotic telomere clustering [GO:0045141]; mismatch repair [GO:0006298]; negative regulation of mitotic recombination [GO:0045950]; nuclear-transcribed mRNA poly(A) tail shortening [GO:0000289]; oogenesis [GO:0048477]; positive regulation of isotype switching to IgA isotypes [GO:0048298]; positive regulation of isotype switching to IgG isotypes [GO:0048304]; resolution of meiotic recombination intermediates [GO:0000712]; response to bacterium [GO:0009617]; somatic hypermutation of immunoglobulin genes [GO:0016446]; spermatogenesis [GO:0007283]
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GO - Molecular function (MF):
ATP binding [GO:0005524]; ATP hydrolysis activity [GO:0016887]; chromatin binding [GO:0003682]; enzyme binding [GO:0019899]; guanine/thymine mispair binding [GO:0032137]...
ATP binding [GO:0005524]; ATP hydrolysis activity [GO:0016887]; chromatin binding [GO:0003682]; enzyme binding [GO:0019899]; guanine/thymine mispair binding [GO:0032137]
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GO - Cellular component (CC):
chiasma [GO:0005712]; chromosome [GO:0005694]; late recombination nodule [GO:0005715]; male germ cell nucleus [GO:0001673]; membrane [GO:0016020]; mismatch repair complex [GO:0032300]; MutLalpha complex [GO:0032389]; nucleoplasm [GO:0005654]; nucleus [GO:0005634]; synaptonemal complex [GO:0000795]...
chiasma [GO:0005712]; chromosome [GO:0005694]; late recombination nodule [GO:0005715]; male germ cell nucleus [GO:0001673]; membrane [GO:0016020]; mismatch repair complex [GO:0032300]; MutLalpha complex [GO:0032389]; nucleoplasm [GO:0005654]; nucleus [GO:0005634]; synaptonemal complex [GO:0000795]
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MLH1
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Kaplan plot for MLH1 in
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*NOTE: the Kaplan plots were collected from OncoLnc