When TDP-43 function is lost, these cryptic exons stay incorporated and often introduce frameshift or premature stop codons, targeting aberrant transcripts for 

2021. 10. 25. · Dendritic spine loss induced by TDP-43 knockdown is rescued by wild-type TDP-43, but not ALS/FTLD-associated mutants, suggesting a common TDP-43 functional deficiency

2014. 2. 1. · TDP-43 is normally expressed in the nucleus of neurons where its most important function is to regulate RNA processing, including mRNA splicing, mainly by binding to UG-rich intronic regions 11, 12.However, in the ALS–FTD patients, TDP-43-positive inclusions are typically found in the neuronal cytoplasm and accompanied by a loss of the normal nuclear expression

TDP-43 is abundantly expressed by all Sox10-positive Schwann cells (Figure 1A, Figure 1—figure supplement 1A, B).To elucidate the PNS-autonomous function of TDP-43, we specifically ablated TDP-43 from Schwann cells by combining the TDP-43 conditional allele (Tardbp fl/fl) (Chiang et al., ) with Dhh-Cre (Jaegle et al., 2003).In these conditional knockout (cKO) mice, TDP-43 expression is

Moreover, the processing body (P-body) marker DCP1a is detected in TDP-43 granules during recovery from stress. Overall, this study supports a central role for 

In relation to its function, TDP-43 has a variety of diverse roles including gene transcription, RNA splicing, RNA shuttling and translation, and microRNA 

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is characterized by selective loss of motor neurons in brain and spinal cord. TAR DNA-binding protein 43 (TDP-43) was identified as a major component of disease pathogenesis in ALS, frontotemporal lobar degeneration (FTLD), and other neurodegenerative disease.

2011. 4. 1. · TDP-43 is an evolutionarily conserved ubiquitously expressed DNA/RNA-binding protein. Although recent studies have shown its association with a variety of neurodegenerative disorders, the function of TDP-43 remains poorly understood. Here we address TDP-43 function using spermatogenesis as a model system.

Thus, denervation of NMJs and dysregulated synaptic function may be related to declining neuronal function in ALS and FTLD. TDP-43 

TDP-43 is a conserved RNA-binding protein with critical roles in splicing in the nervous system 6. TDP-43 also demonstrates tight autoregulation by binding to its transcript, triggering alternative

TDP-43 physiological functions and pathobiology. The protein structure of TDP-43 and its involvement in ALS/. FTLD diseases have already been described in