Telomere length regulation protein TEL2 homolog (TELO2)

Telomere length regulation protein TEL2 homolog (TELO2) is a conserved protein that acts as a key molecular adaptor and regulator of the DNA damage response. It is part of the TTT complex (TELO2-TTI1-TTI2), crucial for the stability and function of the phosphatidylinositol 3-kinase-related protein kinases (PIKKs), which include ATM, ATR, and mTOR. These kinases govern diverse cellular responses, including cell growth, survival, and genome maintenance. While initially characterized for a role in telomere length regulation in yeast, mammalian TELO2 is primarily known for stabilizing PIKK proteins, facilitating the assembly and activity of the mTOR complexes, and contributing to resistance against DNA damage stresses such as ionizing radiation and chemotherapeutic agents. Mutations in TELO2 are associated with syndromes featuring developmental delay, cerebellar atrophy, and seizures. Though critical in oncology-relevant pathways, TELO2 is not currently a direct drug target.

Other names

TELO2CLK2TEL2telomere maintenance 2YHFSKIAA0683protein clk-2 homologhCLK2