| Inheritance | Autosomal dominant |
| Protein | TAR DNA-binding protein 43 (TDP-43) |
| Function | RNA-binding protein involved in RNA splicing, stability, and transport |
| Molecular weight | ~414 amino acids |
| Key domains | N-terminal domain, RNA recognition motif (RRM), C-terminal glycine-rich domain |
| Majority | Located in C-terminal glycine-rich region |
| Effect | Protein mislocalization and aggregation |
| Databases | OMIMOrphanetClinicalTrialsPubMed |