Mus musculus L. (mouse) [MMU]

FULL NAME: DNA mismatch repair protein Mlh1


DESCRIPTION:
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-Msh6) 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 (By similarity).

STRUCTURE SIMILARITY:
Belongs to the DNA mismatch repair mutL/hexB family.


RELATED PATHWAY(S):
Fanconi anemia (FA) pathway
mismatch repair (MMR)


Amino acids sequence

        10         20         30         40         50         60
MAFVAGVIRR LDETVVNRIA AGEVIQRPAN AIKEMIENCL DAKSTNIQVV VKEGGLKLIQ
        70         80         90        100        110        120
IQDNGTGIRK EDLDIVCERF TTSKLQTFED LASISTYGFR GEALASISHV AHVTITTKTA
       130        140        150        160        170        180
DGKCAYRASY SDGKLQAPPK PCAGNQGTLI TVEDLFYNII TRRKALKNPS EEYGKILEVV
       190        200        210        220        230        240
GRYSIHNSGI SFSVKKQGET VSDVRTLPNA TTVDNIRSIF GNAVSRELIE VGCEDKTLAF
       250        260        270        280        290        300
KMNGYISNAN YSVKKCIFLL FINHRLVESA ALRKAIETVY AAYLPKNTHP FLYLSLEISP
       310        320        330        340        350        360
QNVDVNVHPT KHEVHFLHEE SILQRVQQHI ESKLLGSNSS RMYFTQTLLP GLAGPSGEAA
       370        380        390        400        410        420
RPTTGVASSS TSGSGDKVYA YQMVRTDSRE QKLDAFLQPV SSLGPSQPQD PAPVRGARTE
       430        440        450        460        470        480
GSPERATRED EEMLALPAPA EAAAESENLE RESLMETSDA AQKAAPTSSP GSSRKRHRED
       490        500        510        520        530        540
SDVEMVENAS GKEMTAACYP RRRIINLTSV LSLQEEISER CHETLREMLR NHSFVGCVNP
       550        560        570        580        590        600
QWALAQHQTK LYLLNTTKLS EELFYQILIY DFANFGVLRL SEPAPLFDLA MLALDSPESG
       610        620        630        640        650        660
WTEDDGPKEG LAEYIVEFLK KKAEMLADYF SVEIDEEGNL IGLPLLIDSY VPPLEGLPIF
       670        680        690        700        710        720
ILRLATEVNW DEEKECFESL SKECAMFYSI RKQYILEEST LSGQQSDMPG STSKPWKWTV
       730        740        750        760
EHIIYKAFRS HLLPPKHFTE DGNVLQLANL PDLYKVFERC  

Encoded by Mlh1 gene

FULL NAME: mutL homolog 1 (E. coli)


OTHER NAME(S):
1110035C23Rik
AI317206
AI325952
AI561766


Nucleic acid sequence

        10         20         30         40         50         60
atggcgtttg tagcaggagt tattcggcgt ctggacgaga cggtagtgaa ccgcatagcg
        70         80         90        100        110        120
gcgggggaag tcattcagcg gccggccaat gctatcaaag agatgataga aaactgttta
       130        140        150        160        170        180
gatgcaaaat ctacaaatat tcaagtggtt gttaaggaag gtggcctgaa gctaattcag
       190        200        210        220        230        240
atccaagaca atggcactgg aatcaggaag gaagatctgg atattgtgtg tgagaggttc
       250        260        270        280        290        300
actacgagta aactgcagac ttttgaggat ttagccagta tttctaccta tggctttcgt
       310        320        330        340        350        360
ggtgaggcat tggcaagcat aagccatgtg gcccatgtca ctattacaac caaaacagct
       370        380        390        400        410        420
gatgggaaat gtgcgtacag agcaagttac tcagatggaa agctgcaagc ccctcctaaa
       430        440        450        460        470        480
ccctgtgcag gcaaccaggg caccctgatc acggtggaag acctttttta caacataatc
       490        500        510        520        530        540
acaaggagga aagctttaaa aaatccaagt gaagagtacg gaaaaatttt ggaagttgtt
       550        560        570        580        590        600
ggcaggtatt caatacacaa ttcaggcatt agtttctcag ttaaaaaaca aggtgagaca
       610        620        630        640        650        660
gtatctgatg tcagaacact gcccaatgcc acaaccgtgg acaacattcg ctccatcttt
       670        680        690        700        710        720
ggaaatgcgg ttagtcgaga actgatagaa gttgggtgtg aggataaaac cctagctttc
       730        740        750        760        770        780
aaaatgaatg gctatatatc gaatgcaaac tattcagtga agaagtgcat tttcctactc
       790        800        810        820        830        840
ttcatcaacc accgtctggt agaatcagct gccttgagaa aagccattga aactgtatat
       850        860        870        880        890        900
gcagcatact tgcccaaaaa cacacaccca ttcctgtacc tcagtttgga aatcagccct
       910        920        930        940        950        960
cagaacgtgg acgtcaatgt acaccccacc aagcacgaag ttcactttct gcacgaggag
       970        980        990       1000       1010       1020
agcattctgc agcgtgtgca gcagcacatt gagagcaagc tgctgggctc caattcctcc
      1030       1040       1050       1060       1070       1080
aggatgtatt tcacccagac cttgcttcca ggacttgctg ggccttctgg ggaggcagct
      1090       1100       1110       1120       1130       1140
agacccacga caggggtggc ttcctcatcc actagtggaa gtggcgacaa ggtctacgct
      1150       1160       1170       1180       1190       1200
taccagatgg tccgtacgga ctcccgggag cagaagcttg acgcctttct gcagcctgta
      1210       1220       1230       1240       1250       1260
agcagccttg ggcccagcca gccccaggac cctgcccctg tccgaggggc caggacggag
      1270       1280       1290       1300       1310       1320
ggatctcctg aaagggccac gcgggaggac gaggagatgc ttgctctccc agcccccgct
      1330       1340       1350       1360       1370       1380
gaagcagctg ctgagagtga gaacttggag agggaatcac taatggagac ttcagacgca
      1390       1400       1410       1420       1430       1440
gcccagaagg cggcacccac ttccagtcca ggaagctcca gaaagagaca tcgggaggac
      1450       1460       1470       1480       1490       1500
tctgatgtgg aaatggtgga aaatgcttcc gggaaggaaa tgacagctgc ttgctacccc
      1510       1520       1530       1540       1550       1560
aggaggagga tcattaacct caccagcgtc ttgagtctcc aggaagagat tagtgagcgg
      1570       1580       1590       1600       1610       1620
tgccatgaga ctctccggga gatgctccgt aaccattcct ttgtgggctg tgtgaatcct
      1630       1640       1650       1660       1670       1680
cagtgggcct tggcacagca ccagaccaag ctctacctcc tcaacactac caagctcagt
      1690       1700       1710       1720       1730       1740
gaagagctgt tctaccagat actcatttat gattttgcca actttggtgt tctgaggtta
      1750       1760       1770       1780       1790       1800
tcggaaccag cgccactctt cgacctggcc atgctggcct tagacagtcc tgaaagtggc
      1810       1820       1830       1840       1850       1860
tggacagagg acgacggccc gaaggaaggg cttgcagagt acattgttga gtttctgaag
      1870       1880       1890       1900       1910       1920
aagaaagcgg agatgcttgc agactatttc tctgtggaga tcgatgagga agggaacctg
      1930       1940       1950       1960       1970       1980
attggattac ctcttctgat tgacagctat gtgccacctt tggagggact gcctatcttc
      1990       2000       2010       2020       2030       2040
attcttcgac tggccactga ggtgaattgg gatgaagaaa aggagtgttt tgaaagtctc
      2050       2060       2070       2080       2090       2100
agtaaagaat gtgctatgtt ttactccatt cggaagcagt atatactgga ggagtcgacc
      2110       2120       2130       2140       2150       2160
ctctcaggcc agcagagtga catgcctggc tccacgtcaa agccctggaa gtggactgtg
      2170       2180       2190       2200       2210       2220
gagcacatta tctataaagc cttccgctca cacctcctac ctccgaagca tttcacagaa
      2230       2240       2250       2260       2270       2280
gatggcaatg tcctgcagct tgccaacctg ccagatctat acaaagtctt tgagcggtgt

taa     

Last modification date: Oct. 2, 2011