MATR3 (Matrin 3) is a DNA- and RNA-binding protein encoded by the *MATR3* gene, primarily localized in the nuclear matrix. It plays critical roles in maintaining chromatin architecture, transcriptional regulation, and RNA processing/stability. Structurally, MATR3 contains zinc finger domains and nuclear localization signals, enabling interactions with nucleic acids and proteins. Research links MATR3 to neurodegenerative diseases, particularly amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), where pathogenic mutations (e.g., S85C, F115C) disrupt RNA metabolism, induce protein aggregation, and promote neuronal toxicity. MATR3 antibodies are essential tools for studying its expression, localization, and molecular interactions in disease models. They are widely used in techniques like Western blotting, immunofluorescence, and immunoprecipitation to investigate MATR3's role in RNA splicing, stress granule dynamics, and pathological aggregates. Recent studies also explore its involvement in myopathy and cancer, highlighting its diverse cellular functions. Developing high-specificity MATR3 antibodies remains crucial for advancing mechanistic insights and therapeutic targeting in neurodegeneration.