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CHEK2 (CHK2 checkpoint homolog (S. pombe))

Identity

Other namesCHK2
CDS1
Rad53
HGNC (Hugo) CHEK2
Location 22q12.1
Location_base_pair Starts at 27413731 and ends at 27467822 bp from pter ( according to hg18-Mar_2006)  [Mapping]

DNA/RNA

Description 17 exons spanning 57 kb
Transcription Two isoforms are expressed, isoform a (2547nt) includes all 17 exons, while isoform b (2460 nt) does not include exon 12, deleting 87nt (29 codons) from the mRNA. The translation start site is in exon 4.

Protein

Description 61 kDa. Isoform a: 543 amino acids; isoform b: 514 amino acids. Contains FHA and ser/thr kinase domains. Molecular studies of Chk2 typically do not distinguish between the different isoforms.
Expression All tissues tested.
Localisation nuclear
Function Chk2 plays a role in the DNA damage signal cascade, especially in response to double-strand breaks. After detection of DNA damage, Chk2 is phosphorylated on Thr-68 by ATM and ATR. Thus activated, Chk2 targets p53 for phosphorylation on Ser20, releasing p53 from its inhibitor MDM2 and allowing transcriptional activation of genes responsible for cell cycle arrest, such as p21waf1/cip1, as well as initiation of apoptosis. In S phase, Chk2 phosphorylates Cdc25A on Ser123, targeting it for degradation and making it unavailable for the activation of cdk2, thus inhibiting the advance of S phase. In G2 phase, Chk2 phosphorylates Ser216 of Cdc25C, blocking entry into mitosis.

Chk2 is also involved in the regulation of BRCA1. Under normal conditions the two proteins are associated; after irradiation Chk2 phosphorylates Ser988 of BRCA1. This step is required for their dissociation, and the liberated BRCA1 participates directly in DNA repair and cell cycle arrest.

Finally, Chk2 can provoke apoptosis independently of p53, for example via phosphorylation of PML.

Homology 26 % identical to the Rad53 S. cereviscea homolog. The FHA and kinase domains are particularly conserved.

Mutations

Germinal The northern european founder mutation "1100delC" is the most common found in breast cancer families. Other small deletions, stops, and missense mutations in the FHA or kinase domains such as Arg145Trp and Ile157Thr are rare in cancer families but not found in controls. The 1100delC mutation appears to increase the penetrance of mutations in certain other breast cancer genes, notably BRCA2. It should be noted that the publications describing "1100delC" have used the A of the initiation codon as nucleotide 1. This mutation thus corresponds to position 1861 in the complete, isoform a mRNA.
Somatic Missense mutations in the FHA and kinase domains as well as frameshifts and nonsense mutations have been found at low frequencies in osteosarcoma and more rarely in carcinomas of the ovary, lung, and vulva. Reduced or missing protein expression has been observed in some cases of non-Hodgkins lymphoma, although neither mutation nor silencing of the gene by methylation was detected.

Implicated in

Entity Li-Fraumeni, Li-Fraumeni-like syndrome, somatic osteosarcoma and familial aggregations of breast cancer and colon cancer.
Note The importance of Chk2 mutations in hereditary cancer risk is controversial, as some studies have failed to show an excess of mutations in selected populations, such as male breast cancer and patients with multiple colorectal adenomas developing colon cancer. In addition, some studies of breast cancer families suggest that only the relatively frequent 1100delC mutation is significant.
Prognosis No known association with the clinical parameters of solid tumors. There is a possible association with more agressive non-Hodgkins lymphomas.
  

External links

Nomenclature
HGNC (Hugo)CHEK2   16627
Entrez_Gene (NCBI)CHEK2  11200  CHK2 checkpoint homolog (S. pombe)
Cards
AtlasCHEK2ID312
GeneCards (Weizmann)CHEK2
Ensembl (Hinxton)ENSG00000183765 [Gene_View]  CHEK2 [Vega]
AceView (NCBI)CHEK2
Genatlas (Paris)CHEK2
euGene (Indiana)11200
SOURCE (Stanford)NM_001005735 NM_007194 NM_145862
Genomic and cartography
GoldenPath (UCSC)CHEK2  -  22q12.1   chr22:27413731-27467822 -  22q12.1   [Description]    (hg18-Mar_2006)
EnsemblCHEK2 - 22q12.1 [CytoView]
Mapping of homologs : NCBICHEK2 [Mapview]
OMIM114480   176807   259500   604373   609265   
Gene and transcription
Gene : Genbank (Entrez)AB040105 AF086904 AF096279 AF174135 AF217975
Reference sequence (RefSeq transcript) :SRSNM_001005735 NM_007194 NM_145862
Reference transcript : EntrezNM_001005735 NM_007194 NM_145862
RefSeq genomic : SRSAC_000065 AC_000154 NC_000022 NG_008150 NT_011520 NW_001838745 NW_927628
RefSeq genomic : EntrezAC_000065 AC_000154 NC_000022 NG_008150 NT_011520 NW_001838745 NW_927628
Consensus coding sequences : CCDS NCBICHEK2
Cluster EST : UnigeneHs.505297 [ SRS ] Hs.505297 [ NCBI ]
Alternative Splicing : Fast-db (Paris)3445
Protein : pattern, domain, 3D structure
Protein : UniProt/SwissProtO96017 (SRS) O96017 (Expasy) O96017 (Uniprot)
With graphics : InterProO96017
Splice isoforms : VarSplice FASTAO96017(VarSplice FASTA)
Domaine pattern : Prosite (SRS)FHA_DOMAIN (PS50006)    PROTEIN_KINASE_ATP (PS00107)    PROTEIN_KINASE_DOM (PS50011)    PROTEIN_KINASE_ST (PS00108)   
Domain pattern : Prosite (Expaxy)FHA_DOMAIN (PS50006)    PROTEIN_KINASE_ATP (PS00107)    PROTEIN_KINASE_DOM (PS50011)    PROTEIN_KINASE_ST (PS00108)   
Domains : Interpro (SRS)FHA    Prot_kinase_core    Protein_kinase_ATP_bd_CS    Se/Thr_pkinase-rel    Ser_thr_pkin_AS    Ser_thr_pkinase   
Domains : Interpro (EBI)FHA    Prot_kinase_core    Protein_kinase_ATP_bd_CS    Se/Thr_pkinase-rel    Ser_thr_pkin_AS    Ser_thr_pkinase   
Related proteins : CluSTrO96017
Domain families : Pfam SRSFHA (PF00498)    Pkinase (PF00069)   
Domain families : Pfam SangerFHA (PF00498)    Pkinase (PF00069)   
Domain families : Pfam NCBIpfam00498    pfam00069   
Domain families : Smart EMBLFHA (SM00240)S_TKc (SM00220)
Domain structure : Prodom (Prabi Lyon)Prot_kinase (PD000001)   
Blocks (Seattle)O96017
Crystal structure of protein : PDB SRS1GXC    2CN5    2CN8   
Crystal structure of protein : PDBSum1GXC    2CN5    2CN8   
Crystal structure of protein : IMB1GXC    2CN5    2CN8   
Crystal structure of protein : PDB RSDB1GXC    2CN5    2CN8   
HPRD05084
Protein Interaction databases
DIP (DOE-UCLA)O96017
IntAct (EBI)O96017
Polymorphism : SNP, mutations, diseases
Single Nucleotide Polymorphism (SNP) : dbSNP NCBICHEK2
SNP : GeneSNP UtahCHEK2
SNP : HGBaseCHEK2
Genetic variants : HAPMAPCHEK2
Somatic Mutations in Cancer : COSMICCHEK2 
Translocation Breakpoints in Cancer : TICdbCHEK2 
Mutations and Diseases : HGMDCHEK2
Hereditary diseases : OMIM114480    176807    259500    604373    609265   
Hereditary diseases : GENETests114480    176807    259500    604373    609265   
Diseases : Genetic AssociationCHEK2
General knowledge
Homologs : HomoloGeneCHEK2
Homology/Alignments : Family Browser UCSCCHEK2
Phylogenetic Trees/Animal Genes : TreeFamCHEK2
Catalytic activity : Enzyme2.7.11.1 [ Enzyme-Expasy ]   2.7.11.1 [ Enzyme-SRS ]   2.7.11.1 [ IntEnz-EBI ]   2.7.11.1 [ BRENDA ]   2.7.11.1 [ KEGG ]   
Chemical/Protein Interactions : CTD11200
Keywords Ontology : AmiGODNA damage checkpoint  nucleotide binding  magnesium ion binding  protein serine/threonine kinase activity  protein binding  ATP binding  nucleus  protein amino acid phosphorylation  response to DNA damage stimulus  cell cycle  DNA damage response, signal transduction resulting in induction of apoptosis  PML body  transferase activity  
Keywords Ontology : EGO-EBIDNA damage checkpoint  nucleotide binding  magnesium ion binding  protein serine/threonine kinase activity  protein binding  ATP binding  nucleus  protein amino acid phosphorylation  response to DNA damage stimulus  cell cycle  DNA damage response, signal transduction resulting in induction of apoptosis  PML body  transferase activity  
Pathways : BIOCARTAATM Signaling Pathway [Genes]    Role of BRCA1, BRCA2 and ATR in Cancer Susceptibility [Genes]    Cell Cycle: G2/M Checkpoint [Genes]    Regulation of cell cycle progression by Plk3 [Genes]   
Pathways : KEGGCell cycle
Other databases
Probes
Probes : ImagenesCHEK2 Related clones (RZPD - Berlin)
Literature
PubMed261 Pubmed reference(s) in Entrez
PubGeneCHEK2

Bibliography

Linkage of ATM to cell cycle regulation by the Chk2 protein kinase.
Matsuoka S, Huang M, Elledge SJ
Science (New York, N.Y.). 1998 ; 282 (5395) : 1893-1897.
PMID 9836640
 
DNA damage-induced cell cycle checkpoints and DNA strand break repair in development and tumorigenesis.
Dasika GK, Lin SC, Zhao S, Sung P, Tomkinson A, Lee EY
Oncogene. 1999 ; 18 (55) : 7883-7899.
PMID 10630641
 
p53, CHK2, and CHK1 genes in Finnish families with Li-Fraumeni syndrome: further evidence of CHK2 in inherited cancer predisposition.
Vahteristo P, Tamminen A, Karvinen P, Eerola H, Eklund C, Aaltonen LA, Blomqvist C, Aittomˆ§ki K, Nevanlinna H
Cancer research. 2001 ; 61 (15) : 5718-5722.
PMID 11479205
 
Mutations of the CHK2 gene are found in some osteosarcomas, but are rare in breast, lung, and ovarian tumors.
Miller CW, Ikezoe T, Krug U, Hofmann WK, Tavor S, Vegesna V, Tsukasaki K, Takeuchi S, Koeffler HP
Genes, chromosomes & cancer. 2002 ; 33 (1) : 17-21.
PMID 11746983
 
Contribution of the CHEK2 1100delC variant to risk of multiple colorectal adenoma and carcinoma.
Lipton L, Fleischmann C, Sieber OM, Thomas HJ, Hodgson SV, Tomlinson IP, Houlston RS
Cancer letters. 2003 ; 200 (2) : 149-152.
PMID 14568168
 
Variants in CHEK2 other than 1100delC do not make a major contribution to breast cancer susceptibility.
Schutte M, Seal S, Barfoot R, Meijers-Heijboer H, Wasielewski M, Evans DG, Eccles D, Meijers C, Lohman F, Klijn J, van den Ouweland A, Futreal PA, Nathanson KL, Weber BL, Easton DF, Stratton MR, Breast Cancer Linkage Consortium, Rahman N
American journal of human genetics. 2003 ; 72 (4) : 1023-1028.
PMID 12610780
 
CHEK2 1100delC is not a risk factor for male breast cancer population.
Syrjˆ§koski K, Kuukasjˆ§rvi T, Auvinen A, Kallioniemi OP
International journal of cancer. Journal international du cancer. 2004 ; 108 (3) : 475-476.
PMID 14648717
 
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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Contributor(s)

Written05-2004Nancy Uhrhammer

Citation

This paper should be referenced as such :
Uhrhammer N . CHEK2 (CHK2 checkpoint homolog (S. pombe)). Atlas Genet Cytogenet Oncol Haematol. May 2004 .
URL : http://AtlasGeneticsOncology.org/Genes/CHEK2ID312.html

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indexed on : Sat Jun 27 16:39:10 CEST 2009

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