Anti-DNA Ligase IV/LIG4 Antibody Picoband® Fluoro647 Conjugated

DNA ligase 4 is an enzyme that in humans is encoded by the LIG4 gene. The protein encoded by this gene is a DNA ligase that joins single-strand breaks in a double-stranded polydeoxynucleotide in an ATP-dependent reaction. This protein is essential for V (D) J recombination and DNA double-strand break (DSB) repair through nonhomologous end joining (NHEJ) . This protein forms a complex with the X-ray repair cross complementing protein 4 (XRCC4), and further interacts with the DNA-dependent protein kinase (DNA-PK) . Both XRCC4 and DNA-PK are known to be required for NHEJ. The crystal structure of the complex formed by this protein and XRCC4 has been resolved. Defects in this gene are the cause of LIG4 syndrome. Alternatively spliced transcript variants encoding the same protein have been observed.

Plays a role in cell adhesion, and in cohesion of the endothelial monolayer at intercellular junctions in vascular tissue. Its expression may allow melanoma cells to interact with cellular elements of the vascular system, thereby enhancing hematogeneous tumor spread. Could be an adhesion molecule active in neural crest cells during embryonic development. Acts as surface receptor that triggers tyrosine phosphorylation of FYN and PTK2/FAK1, and a transient increase in the intracellular calcium concentration.

1. Ben-Omran, T. I., Cerosaletti, K., Concannon, P., Weitzman, S., Nezarati, M. M. A patient with mutations in DNA ligase IV: clinical features and overlap with Nijmegen breakage syndrome. Am. J. Med. Genet. 137A: 283-287, 2005. 2. Frank, K. M., Sekiguchi, J. M., Seidl, K. J., Swat, W., Rathbun, G. A., Cheng, H.-L., Davidson, L., Kangaloo, L., Alt, F. W. Late embryonic lethality and impaired V (D) J recombination in mice lacking DNA ligase IV. Nature 396: 173-177, 1998. 3. Frank, K. M., Sharpless, N. E., Gao, Y., Sekiguchi, J. M., Ferguson, D. O., Zhu, C., Manis, J. P., Horner, J., DePinho, R. A., Alt, F. W. DNA ligase IV deficiency in mice leads to defective neurogenesis and embryonic lethality via the p53 pathway. Molec. Cell 5: 993-1002, 2000.