Description
Protocol
Available protocols: ELISA+
Specificity / Sensitivity
The PathScan
Species Reactivity: Human, Mouse
Background
TDP43 (TAR DNA-binding protein 43) is involved in transcriptional regulation and exon splicing (1,2). While normal TDP43 is a nuclear protein, pathological TDP43 is a component of insoluble aggregates in patients with frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). In these disorders, TDP43 is abnormally ubiquitinated, phosphorylated, and cleaved to generate carboxy-terminal fragments that are sequestered as insoluble aggregates in neuronal nuclei, perikarya, and neurites (3,4). Additionally, TDP43 inhibits the expression of the gene and regulates gene splicing (1,5).
Alternate Names
ALS10; TADBP; TAR DNA binding protein; TAR DNA-binding protein 43; TAR DNA-binding protein-43; TARDBP; TDP-43; TDP43
