PMS2 Component of the post-replicative DNA mismatch repair system (MMR). Heterodimerizes with MLH1 to form MutL alpha. 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. Belongs to the DNA mismatch repair MutL/HexB family. 4 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Cell cycle regulation; DNA repair, damage; EC 3.1.-.-; Hydrolase; Tumor suppressor
Chromosomal Location of human Ortholog: 7p22.1
Cellular Component:  cytoplasmic ribonucleoprotein granule; cytosol; microtubule cytoskeleton; nucleoplasm; nucleus; plasma membrane
Molecular Function:  ATP binding; DNA binding; endonuclease activity; protein binding; single base insertion or deletion binding
Biological Process:  mismatch repair; nucleic acid phosphodiester bond hydrolysis; response to drug
Disease: Colorectal Cancer, Hereditary Nonpolyposis, Type 4; Mismatch Repair Cancer Syndrome 4
Reference #:  P54278 (UniProtKB)
Alt. Names/Synonyms: DNA mismatch repair protein PMS2; H_DJ0042M02.9; HNPCC4; Mismatch repair endonuclease PMS2; MLH4; PMS1 homolog 2, mismatch repair protein; PMS1 homolog 2, mismatch repair system component; PMS1 protein homolog 2; PMS2; PMS2 postmeiotic segregation increased 2; PMS2 postmeiotic segregation increased 2 (S. cerevisiae); PMS2CL; PMSL2; postmeiotic segregation increased 2 nirs variant 6
Gene Symbols: PMS2
Molecular weight: 95,797 Da
Basal Isoelectric point: 6.4  Predict pI for various phosphorylation states
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

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