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Target intelligence / Profile preview
TELO2-interacting protein 2 (TTI2)
- Target
- TTI2
- Molecular classification
- scaffold/chaperone-associated protein
01
Overview
TELO2-interacting protein 2 (TTI2) is a regulatory protein encoded by the human TTI2 gene, conserved across species and essential for the DNA damage response. As part of the Triple T (TTT) complex, TTI2 regulates the stability and folding of phosphoinositide-3-kinase-related protein kinases (PIKKs), including ATM and TOR. These actions contribute to cell resistance against DNA damage and the maintenance of cell cycle checkpoints. Mutations in TTI2 lead to significant neurodevelopmental disorders, highlighting its importance in brain development and cellular homeostasis. Structural studies reveal that TTI2 forms elongated helical repeats facilitating the proper recruitment of PIKKs to chaperone systems such as R2TP and HSP90, linking protein quality control with DDR signaling[1][2][4][6].
- Other names
- C8orf41MRT39TTI2FLJ23263TELO2 interacting protein 2
02
Mechanism of action
Null
03
Biological functions
DNA damage responseCell cycle checkpoint regulation (intra-S and G2/M checkpoints)Maintenance of PIKK protein abundance
04
Disease associations
Intellectual disabilityMicrocephalyDevelopmental anomalies
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