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Description

Protein PAXX (UniProt: 9BUH6, also known as Paralog of XRCC4 and XLF, XRCC4-like small protein) is encoded by the C9orf142 (also known as PAXX, XLS) gene (Gene ID: 286257) in human. PAXX is a newer member of XRCC4 superfamily that predominantly localizes to the nucleus and is shown to accumulate at sites of DNA damage generated by laser microirradiation. It is involved in non-homologous end joining (NHEJ), a major pathway to repair double-strand breaks in DNA and may act as a scaffold required to stabilize the Ku heterodimer, composed of XRCC5/Ku80 and XRCC6/Ku70, at double-strand break sites where it promote the assembly and/or stability of the NHEJ machinery. PAXX residues 1-113 form a head domain, which is structurally related to those of XRCC4, XLF and SAS6. This is followed by a 31-amino-acid -helix, forming a coiled-coil with the other protomer to make a PAXX homodimer. Two isoforms of PAXX have been described that are produced by alternative splicing. (Ref.: Ochi, T., et al. (2015). Science 347(6218), 185-188).