DNA cross-link repair 1A protein is a protein that in humans is encoded by the DCLRE1Agene.[1][2][3]
DNA interstrand cross-links prevent strand separation, thereby physically blocking transcription, replication, and segregation of DNA. DCLRE1A is one of several evolutionarily conserved genes involved in repair of interstrand cross-links (Dronkert et al., 2000).[supplied by OMIM][3]
Function
The protein DCLRE1A (DNA cross-link repair 1A) is also referred to as SNM1A (sensitive to nitrogen mustard 1A). DCLRE1A is a 5’ to 3’ exonuclease that forms a complex with the Cockayne syndrome B (CSB) protein. In this complex, CSB modulates the exonuclease activity of DCLRE1A and coordinates the efficient assembly of DCLRE1A to sites of DNA damage.[4] In human cells, this complex is recruited to DNA inter-strand cross-links, a form of DNA damage. The complex then participates in the repair of the cross-linked DNA. DCLRE1A protein is thought to be recruited by CSB to facilitate cross-link unhooking following incision 5’ to the cross-link by another complex, the ERCC1/XPF nuclease complex.[4] Failure of the DCLRE1A/CSB complex to carry out its repair function may contribute to the degenerative pathologies and premature aging features of Cockayne syndrome.
References
↑Demuth I, Digweed M (Nov 1998). "Genomic organization of a potential human DNA-crosslink repair gene, KIAA0086". Mutat Res. 409 (1): 11–6. doi:10.1016/s0921-8777(98)00037-8. PMID9806498.
Nagase T, Miyajima N, Tanaka A, et al. (1995). "Prediction of the coding sequences of unidentified human genes. III. The coding sequences of 40 new genes (KIAA0081-KIAA0120) deduced by analysis of cDNA clones from human cell line KG-1". DNA Res. 2 (1): 37–43. doi:10.1093/dnares/2.1.37. PMID7788527.
Zhang X, Richie C, Legerski RJ (2003). "Translation of hSNM1 is mediated by an internal ribosome entry site that upregulates expression during mitosis". DNA Repair (Amst.). 1 (5): 379–90. doi:10.1016/S1568-7864(02)00015-0. PMID12509242.
Deloukas P, Earthrowl ME, Grafham DV, et al. (2004). "The DNA sequence and comparative analysis of human chromosome 10". Nature. 429 (6990): 375–81. doi:10.1038/nature02462. PMID15164054.