BRCA1-associated ATM activator 1 (BRAT1)

BRCA1-associated ATM activator 1 (BRAT1) is a scaffolding protein critical for the proper assembly and function of the Integrator complex, which is involved in the 3’ end processing of various noncoding RNAs and pre-mRNAs[1][2][3]. BRAT1 tightly interacts with the INTS9/INTS11 subunits, facilitating Integrator cleavage module maturation, nuclear import, and stabilization; it is also required for activating key neuronal genes during differentiation by modulating REST occupancy and interacting with the RNA polymerase II complex[1][2][3]. Mutations in BRAT1 are linked to a spectrum of neurodevelopmental and neurodegenerative disorders characterized by impaired RNA processing, altered gene expression, and defective neuronal differentiation[1][2][3]. Loss of BRAT1 leads to misprocessing of small nuclear and nucleolar RNAs, deformation of nuclear structures such as Cajal bodies, and destabilization of the Integrator complex, particularly impacting neural development and function[1][2][3]. There is currently no evidence that BRAT1 is a direct therapeutic target or that any approved drugs act on it.

Other names

Integrator complex assembly factor BRAT1BAAT1C7orf27MGC22916BRCA1-associated protein required for ATM activation protein 1NEDCASRMFSLHEAT repeat-containing protein C7orf27