Recombinant Human Matrin-3 (MATR3), Truncated

Uniprot Id

P43243

Target Species

Human

Target Name

MATR3

Target Full Name

Matrin-3

Target 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. May bind to specific miRNA hairpins.

Target Involvement

Amyotrophic lateral sclerosis 21 (ALS21)

Target Subcellular Location

Nucleus matrix.

Target Synonyms

ALS21; KIAA0723; Matr3; MATR3_HUMAN; Matrin-3; Matrin3; MPD2; VCPDM; Vocal cord and pharyngeal weakness with distal myopathy

Target Background

This gene encodes a nuclear matrix protein, which is proposed to stabilize certain messenger RNA species. Mutations of this gene are associated with distal myopathy 2, which often includes vocal cord and pharyngeal weakness. Alternatively spliced transcript variants, including read-through transcripts composed of the upstream small nucleolar RNA host gene 4 (non-protein coding) and matrin 3 gene sequence, have been identified. Pseudogenes of this gene are located on chromosomes 1 and X.