tdp-43 function

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

An acetylation switch controls TDP-43 function and aggregation propensity TDP-43 pathology is a disease hallmark that characterizes amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD-TDP). Although a critical role for TDP-43 as an RNA-binding protein has emerged, the regulation of TDP-43 function is poorly understood.

Accordingly, TDP-43 myogranules have been shown to provide essential functions during skeletal muscle development and regeneration, both in 

These results suggest that functions associated with RRM-1, such as (UG)n RNA binding, confer TDP-43 mobility in the nucleus. The function of RRM-2 remains 

auto-feedback mechanisms, which involve TDP-43 binding to its own 3' untranslated region [15,22]. Overexpression of TDP-43 leads to down-regulation of the endogenous TDP-43 [23,24], and blocking expression of one allele leads to a compensatory increase in the expression of the other allele [25-27]. The tight regulation of TDP-43 levels is

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. We previously showed that TDP-43 binds to the testis

2022. 5. 2. · The pathogenesis of TDP-43 includes the theory of function gain and the theory of function loss [].According to the theory of function gain, overexpression or mutation of TARDBP and mislocalization of TDP-43 can induce toxicity, resulting in neuronal damage and axonal damage (Fig. 2) [32, 33].Neuropathology is characterized by the formation of cytoplasmic TDP

Plays a role in maintaining mitochondrial homeostasis by regulating the processing of mitochondrial transcripts (PubMed:28794432). Regulates also mRNA stability 

Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats ( ) Jianbin Tong et al. EMBO JOURNAL Premature death of TDP-43 (A315T) transgenic mice due to gastrointestinal complications prior to development of full neurological symptoms of amyotrophic lateral sclerosis

Phosphorylated TDP-43 potentiates a number of neurotoxic effects including reduced liquid–liquid phase separation dynamicity, changes in 

2. Genetics, protein structures, and biological functions of TDP-43. TDP-43 was identified from a genomic screen for novel transcriptional inactivators that bind to the TAR-DNA element of the HIV-1 virus, where it functions as a transcriptional repressor [].The human TDP-43 gene, which is located on chromosome 1 and contains 6 exons, is alternatively spliced to generate at least