TDP-43 proteinopathies: a new wave of neurodegenerative diseases
This review highlights the key physiological functions of the TDP-43 protein, while considering an expanding spectrum of neurodegenerative diseases associated with pathogenic TDP-43 deposition, and dissecting key molecular pathways through which TDP-43 may mediate neurodegeneration. http://creativecommons.org/licenses/by-nc/4./
TDP-43: A Key Therapeutic Target beyond Amyotrophic Lateral Sclerosis Accumulation of TDP-43 in the cytoplasm of diseased neurons is the pathological hallmark of frontotemporal dementia-TDP (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), two diseases that lack efficacious medicine to prevent or to stop disease progression.
TDP-43 in aging and Alzheimer's disease - a review - PubMed
transactive response dna-binding protein of 43 kda (tdp-43), an rna and dna binding protein involved in transcriptional repression, rna splicing and rna metabolism during the stress response, is the major component of neuronal inclusions in amyotrophic lateral sclerosis (als) and frontotemporal lobar degeneration with ubiquitin inclusions, now
TDP-43 is a 414 amino acids long, 43 kDa heavy protein. From N-term to C-term one can delimit. In December the structure of TDP-43 was resolved with cryo-EM   but shortly after it was argued that in the context of FTLD-TDP the protein involved could be TMEM106B (which has been also resolved with cryo-EM), rather than of TDP-43.
Triad of TDP43 control in neurodegeneration: autoregulation
Cytoplasmic aggregation of TAR DNA-binding protein 43 (TDP43; also known as TARDBP or TDP-43) is a key pathological feature of several neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). TDP43 typically resides in the nucleus but can shuttle b
TDP-43 as a possible biomarker for frontotemporal lobar degeneration: a
The primary objective of this systematic review is to identify which antibodies have previously been described to detect TDP-43 pathology. These antibodies are expected to be suitable for defining the characteristic profile of pathological TDP-43 in human brain and biofluids, using immunostaining and immunoblotting.
TDP-43 represses cryptic exon inclusion in the FTD-ALS gene UNC13A
Main. TDP-43, encoded by the TARDBP gene, is an abundant, ubiquitously expressed RNA-binding protein that normally localizes to the nucleus. It has a role in fundamental RNA-processing activities, including RNA transcription, alternative splicing and RNA transport 7.A major splicing regulatory function of TDP-43 is to repress the inclusion of cryptic exons during splicing 2,8-10.
TDP-43 as a potential biomarker for amyotrophic lateral sclerosis
Jun 28, · the accumulation of an rna-binding protein, tdp-43, is the most significant pathological finding in approximately 95% of als cases and 50% of ftd cases, and discovery of this common pathological signature, together with an increasing understanding of the shared genetic basis of these disorders, has led to ftd and als being considered as part of a
TDP-43 as a potential biomarker for amyotrophic lateral ... - PubMed
Jun 28, · TDP-43 as a potential biomarker for amyotrophic lateral sclerosis: a systematic review and meta-analysis Authors Vivek Majumder 1 , Jenna M Gregory 2 3 , Marcelo A Barria 4 , Alison Green 4 , Suvankar Pal 5 6 7 Affiliations 1 Centre for Clinical Brain Sciences, University of Edinburgh, Chancellor's Building, Edinburgh, EH16 4SB, UK.
The Different Faces of the TDP-43 Low-Complexity Domain: The Formation
1. Introduction. Transactive response DNA-binding protein 43 (TDP-43) is a nucleic acid-binding protein that is involved in RNA processing and is essential for the development of the central nervous system [1,2].While many studies have elucidated the pivotal roles of TDP-43 in multiple cellular functions, emerging studies have also uncovered its pathological roles after it was identified as