Importin α/β and the tug of war to keep TDP‐43 in solution: quo vadis?
Sign Up to like & getrecommendations! Published in 2022 at "BioEssays"
DOI: 10.1002/bies.202200181
Abstract: The transactivation response‐DNA binding protein of 43 kDa (TDP‐43) is an aggregation‐prone nucleic acid‐binding protein linked to the etiology of Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD). These conditions feature the accumulation of… read more here.
Keywords: tug war; aggregation; keep tdp; importin tug ... See more keywords
Plasma TDP-43 Reflects Cortical Neurodegeneration and Correlates with Neuropsychiatric Symptoms in Huntington’s Disease
Sign Up to like & getrecommendations! Published in 2022 at "Clinical Neuroradiology"
DOI: 10.1007/s00062-022-01150-5
Abstract: Huntington’s disease (HD) is a monogenic neurodegenerative disease with no effective treatment currently available. The pathological hallmark of HD is the aggregation of mutant huntingtin in the medium spiny neurons of the striatum, leading to… read more here.
Keywords: huntington disease; plasma tdp; tdp; neuropsychiatric symptoms ... See more keywords
Experimental analysis of the onset mechanism of TdP reported in an LQT3 patient during pharmacological treatment with serotonin–dopamine antagonists against insomnia and nocturnal delirium
Sign Up to like & getrecommendations! Published in 2019 at "Heart and Vessels"
DOI: 10.1007/s00380-019-01521-y
Abstract: Torsade de pointes (TdP) occurred in a long QT syndrome type 3 (LQT3) patient after switching perospirone to blonanserin. We studied how their electropharmacological effects had induced TdP in the LQT3 patient. Perospirone hydrochloride (… read more here.
Keywords: heart; perospirone; tdp; total peripheral ... See more keywords
Co-occurrence of mixed proteinopathies in late-stage Huntington’s disease
Sign Up to like & getrecommendations! Published in 2017 at "Acta Neuropathologica"
DOI: 10.1007/s00401-017-1786-7
Abstract: Accumulating evidence highlights the potential role of mixed proteinopathies (i.e., abnormal protein aggregation) in the development of clinical manifestations of neurodegenerative diseases (NDD). Huntington’s disease (HD) is an inherited NDD caused by autosomal-dominant expanded CAG… read more here.
Keywords: aggregation; mixed proteinopathies; tdp; huntington disease ... See more keywords
Nucleo-cytoplasmic transport of TDP-43 studied in real time: impaired microglia function leads to axonal spreading of TDP-43 in degenerating motor neurons
Sign Up to like & getrecommendations! Published in 2018 at "Acta Neuropathologica"
DOI: 10.1007/s00401-018-1875-2
Abstract: Transactivating DNA-binding protein-43 (TDP-43) deposits represent a typical finding in almost all ALS patients, more than half of FTLD patients and patients with several other neurodegenerative disorders. It appears that perturbation of nucleo-cytoplasmic transport is… read more here.
Keywords: tdp; motor neurons; transport; nucleo cytoplasmic ... See more keywords
Subcortical TDP-43 pathology patterns validate cortical FTLD-TDP subtypes and demonstrate unique aspects of C9orf72 mutation cases
Sign Up to like & getrecommendations! Published in 2019 at "Acta Neuropathologica"
DOI: 10.1007/s00401-019-02070-4
Abstract: Frontotemporal lobar degeneration with TDP-43 immunoreactive (TDP-ir) inclusions (FTLD-TDP) is sub-classified based on the pattern of neocortical pathology, with each subtype showing clinical and genetic correlations. Recent studies indicate that accurate subtyping of cases may… read more here.
Keywords: tdp; subcortical tdp; pathology; ftld tdp ... See more keywords
TDP-43 transports ribosomal protein mRNA to regulate axonal local translation in neuronal axons
Sign Up to like & getrecommendations! Published in 2020 at "Acta Neuropathologica"
DOI: 10.1007/s00401-020-02205-y
Abstract: Mislocalization and abnormal deposition of TDP-43 into the cytoplasm (TDP-43 proteinopathy) is a hallmark in neurons of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). However, the pathogenic mechanism of the diseases linked to… read more here.
Keywords: neuronal axons; protein; local translation; tdp ... See more keywords
Antibody against TDP-43 phosphorylated at serine 375 suggests conformational differences of TDP-43 aggregates among FTLD–TDP subtypes
Sign Up to like & getrecommendations! Published in 2020 at "Acta Neuropathologica"
DOI: 10.1007/s00401-020-02207-w
Abstract: Aggregation of hyperphosphorylated TDP-43 is the hallmark pathological feature of the most common molecular form of frontotemporal lobar degeneration (FTLD–TDP) and in the vast majority of cases with amyotrophic lateral sclerosis (ALS–TDP). However, most of… read more here.
Keywords: tdp; als tdp; pathology; ftld tdp ... See more keywords
Signature laminar distributions of pathology in frontotemporal lobar degeneration
Sign Up to like & getrecommendations! Published in 2022 at "Acta Neuropathologica"
DOI: 10.1007/s00401-021-02402-3
Abstract: Frontotemporal lobar degeneration (FTLD) with either tau (FTLD-tau) or TDP-43 (FTLD-TDP) inclusions are distinct proteinopathies that frequently cause similar frontotemporal dementia (FTD) clinical syndromes. FTD syndromes often display macroscopic signatures of neurodegeneration at the level… read more here.
Keywords: pathology; layer pathology; tdp; ftld tau ... See more keywords
Mutant TDP-43 Expression Triggers TDP-43 Pathology and Cell Autonomous Effects on Primary Astrocytes: Implications for Non-cell Autonomous Pathology in ALS
Sign Up to like & getrecommendations! Published in 2020 at "Neurochemical Research"
DOI: 10.1007/s11064-020-03048-5
Abstract: Motor neuron degeneration in amyotrophic lateral sclerosis (ALS) caused by mutations in superoxide dismutase 1 (SOD1) is partly non-cell autonomous, involving cellular dysfunction of astrocytes. Whether non-cell autonomous effects occur in other forms of ALS,… read more here.
Keywords: mutant tdp; tdp; non cell; pathology ... See more keywords
Molecular chaperone HSP70 prevents formation of inclusion bodies of the 25-kDa C-terminal fragment of TDP-43 by preventing aggregate accumulation
Sign Up to like & getrecommendations! Published in 2018 at "Cell Stress and Chaperones"
DOI: 10.1007/s12192-018-0930-1
Abstract: Transactive response DNA/RNA-binding protein 43-kDa (TDP-43) C-terminal fragments, such as a 25-kDa fragment (TDP-25), have been identified as a ubiquitinated and phosphorylated components of inclusion bodies (IBs) in motor neurons from amyotrophic lateral sclerosis patients.… read more here.
Keywords: inclusion bodies; hsp70; ibs; tdp ... See more keywords