Matrin-3 (MATR3)

Matrin-3 is a highly conserved 125 kDa protein encoded by the MATR3 gene, widely expressed in human tissues with highest levels in the brain[1][4]. Localized to the nuclear matrix, it is integral to nuclear architecture and contains two RNA recognition motifs and two C2H2 zinc finger domains, allowing it to bind both RNA and DNA[1][4][7]. Matrin-3 is involved in diverse nuclear processes, including RNA stabilization and processing, DNA binding, chromatin organization, gene expression regulation, and DNA repair[2][3][4][7]. Pathogenic mutations in MATR3 are linked to familial forms of amyotrophic lateral sclerosis and distal myopathy[5][6][7]. The protein is not a canonical therapeutic target such as a receptor, ion channel, or enzyme, but it plays critical regulatory roles in normal physiology and disease through its effects on nuclear structure and gene regulation[1][2][3][4][7].

Other names

Matrin 3Nuclear matrix protein Matrin-3NMP-180