TAR DNA-binding protein 43 - Wikipedia
网页TDP-43 is a transcriptional repressor that binds to chromosomally integrated TAR DNA and represses HIV-1 transcription. In addition, this protein regulates alternate splicing of the CFTR gene. In particular, TDP-43 is a splicing factor binding to the intron8/exon9 junction of the CFTR gene and to the intron2/exon3 region of the apoA-II gene.
The role of TDP-43 propagation in neurodegenerative diseases ...
网页2020年10月13日 · The accumulation of TDP-43 aggregates in the central nervous system is a common feature of many neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), frontotemporal dementia ...
TDP-43 proteinopathies: a new wave of neurodegenerative diseases
网页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.
TDP-43 forms amyloid filaments with a distinct fold in type A FTLD-TDP
网页2023年8月2日 · Cryo-electron microscopy structures and mass spectrometry analyses show that TAR DNA-binding protein 43 (TDP-43) forms amyloid filaments with a distinct fold in type A frontotemporal lobar ...
New mechanistic insights into TDP-43 pathology - Nature
网页2023年8月22日 · Two new studies have provided important mechanistic insights into TDP-43 pathology, a hallmark of neurodegenerative conditions such as amyotrophic lateral sclerosis and frontotemporal lobar ...
TDP-43 proteinopathies: a new wave of neurodegenerative diseases
网页2020年11月11日 · Putative mechanisms by which TAR DNA-binding protein 43 (TDP-43) exerts its pathogenic effects in TDP-43 proteinopathies. Multiple processes have been implicated in TDP-43 pathogenesis. Nuclear depletion of TDP-43 results in dysregulation of RNA metabolism resulting in reduced protein production.
A robust evaluation of TDP-43, poly GP, cellular pathology and …
网页TDP-43 and RanGTPase are functional in the C9ORF72 (G 4 C 2) 66 mouse model. TDP-43 dysfunction is a hallmark feature in ALS, and several mouse models of ALS have recapitulated aggregated TDP-43 pathology in situ [45, 63]. We assessed the C9ORF72 (G 4 C 2) 66 mouse model for indicators of TDP-43 dysfunction.
Mechanisms underlying TDP-43 pathology and …
网页2023年3月9日 · TAR DNA binding protein 43 kDa (TDP-43) is a highly conserved ubiquitously expressed nuclear protein that plays an important role in several essential cell functions, including transcriptional repression, RNA splicing, mRNA transport, microRNA maturation, translational regulation, and the formation of stress granules (Sephton et al., …
CUTS RNA Biosensor for the Real-Time Detection of TDP-43 Loss …
网页2024年9月12日 · TDP-43 homotypic phase transitions induce loss of splicing function via CUTS detection. eLife assessment. Recent studies have demonstrated that depletion of nuclear TDP-43 leads to loss of its nuclear function resulting in changes in gene expression and splicing of target mRNAs. This study developed a sensitive and robust sensor for …
科学家揭示TDP-43蛋白参与神经退行性疾病的分子机制
网页2023年11月27日 · TDP-43是一个多功能的DNA和RNA结合蛋白，由TARDBP基因编码，在细胞内的RNA转录、选择性剪接及mRNA稳定性调节等过程中发挥功能。. 在大多数肌萎缩侧索 ...
跳至内容