May play a role in transcription or may interact with other nuclear matrix proteins to form the internal fibrogranular network. In association with the SFPQ-NONO heteromer may play a role in nuclear retention of defective RNAs. Plays a role in the regulation of DNA virus-mediated innate immune response by assembling into the HDP-RNP complex, a complex that serves as a platform for IRF3 phosphorylation and subsequent innate immune response activation through the cGAS-STING pathway.
基因名
MATR3
蛋白名
Matrin-3
亚基
Part of complex consisting of SFPQ, NONO and MATR3. Interacts with EIF2C1 and EIF2C2.
亚细胞定位
Nucleus matrix.
疾病
Defects in MATR3 are the cause of myopathy distal type 2 (MPD2) [MIM:606070]; also called vocal cord and pharyngeal dysfunction with distal myopathy (VCPDM). MPD2 is a muscular disorder characterized by distal weakness, with onset in hands and feet, associated with vocal cord and pharyngeal weakness causing a nasal voice and swallowing disorders.
May play a role in transcription or may interact with other nuclear matrix proteins to form the internal fibrogranular network. In association with the SFPQ-NONO heteromer may play a role in nuclear retention of defective RNAs.