{Reference Type}: Journal Article
{Title}: Elevated CXorf67 Expression in PFA Ependymomas Suppresses DNA Repair and Sensitizes to PARP Inhibitors.
{Author}: Han J;Yu M;Bai Y;Yu J;Jin F;Li C;Zeng R;Peng J;Li A;Song X;Li H;Wu D;Li L;
{Journal}: Cancer Cell
{Volume}: 38
{Issue}: 6
{Year}: 12 2020 14
{Factor}: 38.585
{DOI}: 10.1016/j.ccell.2020.10.009
{Abstract}: Ependymoma is the third most common pediatric tumor with posterior fossa group A (PFA) being its most aggressive subtype. Ependymomas are generally refractory to chemotherapies and thus lack any effective treatment. Here, we report that elevated expression of CXorf67 (chromosome X open reading frame 67), which frequently occurs in PFA ependymomas, suppresses homologous recombination (HR)-mediated DNA repair. Mechanistically, CXorf67 interacts with PALB2 and inhibits PALB2-BRCA2 interaction, thereby inhibiting HR repair. Concordantly, tumor cells with high CXorf67 expression levels show increased sensitivity to poly(ADP-ribose) polymerase (PARP) inhibitors, especially when combined with radiotherapy. Thus, our findings have revealed a role of CXorf67 in HR repair and suggest that combination of PARP inhibitors with radiotherapy could be an effective treatment option for PFA ependymomas.