@article{oai:shiga-med.repo.nii.ac.jp:00004378,
 author = {漆谷, 真 and TAMAKI, Yoshitaka and URUSHITANI, Makoto},
 journal = {International Journal of Molecular Sciences},
 month = {2022-11-21},
 note = {application/pdf, TAR DNA binding protein 43 (TDP-43) is a DNA/RNA binding protein involved in pivotal cellular functions, especially in RNA metabolism. Hyperphosphorylated and ubiquitinated TDP-43-positive neuronal cytoplasmic inclusions are identified in the brain and spinal cord in most cases of amyotrophic lateral sclerosis (ALS) and a substantial proportion of frontotemporal lobar degeneration (FTLD) cases. TDP-43 dysfunctions and cytoplasmic aggregation seem to be the central pathogenicity in ALS and FTLD. Therefore, unraveling both the physiological and pathological mechanisms of TDP-43 may enable the exploration of novel therapeutic strategies. This review highlights the current understanding of TDP-43 biology and pathology, describing the cellular processes involved in the pathogeneses of ALS and FTLD, such as post-translational modifications, RNA metabolism, liquid-liquid phase separation, proteolysis, and the potential prion-like propagation propensity of the TDP-43 inclusions., Journal Article},
 title = {Molecular Dissection of TDP-43 as a Leading Cause of ALS/FTLD},
 year = {}
}