MATR3 : matrin 3
Description
The MATR3 (matrin 3) is a protein-coding gene located on chromosome 5.
The MATR3 gene provides instructions for making a protein called matrin 3, which is found in the nucleus of the cell as part of the nuclear matrix. The nuclear matrix is a network of proteins that provides structural support for the nucleus and aids in several important nuclear functions. The function of the matrin 3 protein is unknown. This protein can attach to (bind) RNA, which is a chemical cousin of DNA. Some studies indicate that matrin 3 binds and stabilizes a type of RNA called messenger RNA (mRNA), which provides the genetic blueprint for proteins. Matrin 3 may also bind certain abnormal RNAs that could lead to nonfunctional or harmful proteins, thereby blocking the formation of such proteins. Other studies suggest that the matrin 3 protein may be involved in cell survival.
Matrin-3 may play a role in transcription or interact with other nuclear matrix proteins to form the internal fibrogranular network. In association with the SFPQ-NONO heteromer, it may play a role in nuclear retention of defective RNAs. It participates in regulating the DNA virus-mediated innate immune response by assembling into the HDP-RNP complex, which serves as a platform for IRF3 phosphorylation and subsequent innate immune response activation through the cGAS-STING pathway. Matrin-3 binds to N6-methyladenosine (m6A)-containing mRNAs and contributes to MYC stability by binding to m6A-containing MYC mRNAs. It may bind to specific miRNA hairpins.
MATR3 is also known as ALS21, MPD2, VCPDM.
Associated Diseases
- Amyotrophic lateral sclerosis
- Amyotrophic lateral sclerosis 21
- Vocal cord and pharyngeal distal myopathy
- Distal myopathy 2
