Anti-Human Matrin-3 Antibody Pair

Product Description: Matrin 3 (MATR3) is a nuclear matrix protein encoded by the MATR3 gene, primarily localized in the nucleus where it contributes to nuclear organization and structural support. It is an intrinsically disordered RNA-binding protein involved in various aspects of nucleic acid metabolism, including transcription regulation, alternative splicing, mRNA stabilization and export, nuclear retention of hyperedited RNA, and RNA silencing and degradation. MATR3 interacts with numerous RNA processing proteins, such as TDP-43 and FUS, and is thought to play a role in repressing cryptic exon inclusions and regulating the DNA damage response. Mutations in MATR3, notably the Ser85Cys (S85C) variant, are linked to distal myopathy 2, characterized by muscle and vocal cord weakness, and have also been implicated in neurodegenerative diseases like amyotrophic lateral sclerosis (ALS). Protein Function: 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 (PubMed:28712728). Binds to N6-methyladenosine (m6A)-containing mRNAs and contributes to MYC stability by binding to m6A-containing MYC mRNAs (PubMed:32245947). May bind to specific miRNA hairpins (PubMed:28431233) [The Uniprot Consortium]