Summary

Name MLH1
Full Name DNA mismatch repair protein Mlh1
Synonyms MutL protein homolog 1 | COCA2
Primary ID P40692
Links - -
Type protein
Relations 4
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.

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Relations

Regulator Mechanism target score ℹ
+ MLH1form complex img/form-complex.png binding MLH1/PMS1 0.691
Publications: 1 Organism: Homo Sapiens
+ TP53up-regulates quantity img/direct-activation.png transcriptional regulation MLH1 0.596
Publications: 1 Organism: Homo Sapiens
+ MLH1form complex img/form-complex.png binding MLH1/PMS2 0.742
Publications: 1 Organism: Homo Sapiens
+ MLH1up-regulates activity img/indirect-activation.png DNA_repair 0.7
Publications: 1 Organism: Mus Musculus