FTLD-TDP
FTLD-TDP with motor neuron disease, visuospatial impairment and a progressive supranuclear palsy-like syndrome: broadening the clinical phenotype of TDP-43 proteinopathies. A report of three cases
... temporal atrophy on MRI (Figure 2) was oriented more towards FTLD; however all displayed considerable fea- tures of PSP. It is noteworthy, that in addition to sub- cortical dementia, typical for PSP [22], all of ...
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Classification of FTLD-TDP cases into pathological subtypes using antibodies against phosphorylated and non-phosphorylated TDP43
... the FTLD-TDP subtypes using the recently updated classification system for FTLD- TDP pathology ...for FTLD-TDP subtyping [3] as illus- trated and detailed in Figure ...
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Spatial patterns of phosphorylation-dependent TDP-43-immunoreactive neuronal cytoplasmic inclusions (NCI) in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)
... sporadic FTLD- ...in FTLD-TDP with GRN mutation (Beck et ...2006). TDP- 43 is a nuclear protein but in FTLD-TDP, TDP-43 is redistributed from the nucleus to the cytoplasm, ...
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C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation
... in FTLD-TDP type C cases as de- scribed by Katsuse and Dickson [26] (6) long thick DNs in cortex in FTLD-TDP type C cases, (7) granular pre- inclusions as described by Katsuse and Dickson ...
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A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)
... include TDP-43 IHC, quantitative pathological information based on TDP-43 IHC may have added complexity or noise to the ...of FTLD-TDP cases to assess inter- rater reliability in assigning the ...
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Investigating the role of filamin C in Belgian patients with frontotemporal dementia linked to GRN deficiency in FTLD-TDP brains
... with FTLD-TDP brain pathology but no mutation in any of the known causal FTD genes, and 1 patient with a mixed Alzheimer’s disease (AD) and FTLD-TDP brain ...
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The role of gene variants in the pathogenesis of neurodegenerative disorders as revealed by next generation sequencing studies: a review
... of FTLD-TDP are mutations in progranulin gene (GRN) and hexanucleo- tide repeat expansion in ...with FTLD-TDP; in other words, homozygosity in the minor allele was protective (reviewed in ...
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Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis
... of FTLD were previously referred to as atypical FTLD with ubiquitinated inclusions (aFTLD-U), neur- onal intermediate filament inclusion disease (NIFID) and basophilic inclusion body disease (BIBD) ...the ...
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Znf179 E3 ligase-mediated TDP-43 polyubiquitination is involved in TDP-43- ubiquitinated inclusions (UBI) (+)-related neurodegenerative pathology
... addition, TDP-43 has also been implicated to mediate spine for- mation and maturation during brain development ...[49]. TDP-43 is a negative regulator of protrusion/spine generation, and the elevated level ...
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Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene
... termed FTLD-tau ...protein, TDP-43, is present within NCI, neuritic processes (dys- trophic neurites, DN) or neuronal intranuclear inclusions (NII) [1, 26]; such cases are collectively termed FTLD- ...
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Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer’s disease and cerebrovascular disease pathologies
... with TDP-43 pathology in aged individuals, we studied data from the National Alzheimer ’ s Coordinating Center (NACC) Neuropathology Data ...available TDP-43 pathology information, sourced from 27 different ...
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Expression of TMEM106B, the frontotemporal lobar degeneration-associated protein, in normal and diseased human brain
... (+) FTLD-TDP cases ex- hibit significantly different patterns of TMEM106B ex- pression is ...(−) FTLD-TDP cases with similar patterns of TDP- 43 pathology did not demonstrate TMEM106B ...
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Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy:effects of genetic, demographic and neuropathological variables
... AD varied between time intervals and the time-de- pendent covariate was significant (t = 2.23, p < 0.05), thus violating the assumption of proportionality, and, second, numbers of patients were small. Bowen et al. ...
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Novel monoclonal antibodies to normal and pathologically altered human TDP-43 proteins
... Murine MAbs were raised against human rTDP-43 pro- teins using similar methods described previously [15-18]. Mice were immunized with FL-rTDP-43 or Nt-rTDP-43. Briefly, rTDP-43 proteins were emulsified with Freund’s ...
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Pathological phosphorylation of tau and TDP-43 by TTBK1 and TTBK2 drives neurodegeneration
... and TDP-43 kinases indicates a possible shared mechanism for the initiation of TDP-43 proteinopathy and tauopathy in ...phosphorylated TDP-43 or tau in the majority of FTLD cases, and the ...
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Distinct TDP-43 inclusion morphologies in frontotemporal lobar degeneration with and without amyotrophic lateral sclerosis
... of TDP-43 inclusion morphologies in predilection cortical regions implicated in FTLD, FTLD-ALS and ALS cases demonstrates a significant amount of circumferential TDP-43 inclusions in the ...
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Therapeutic effect of berberine on TDP-43-related pathogenesis in FTLD and ALS
... 5]. TDP-43 is highly con- served in various species including mammals, flies and caenorhabditis elegans [4, 6] and ubiquitously ex- presses in all tissues including brain [4, ...of TDP-43 ...of ...
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RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy
... between TDP-43 protein and RNA interaction, which prevents splicing of target RNA and increases TDP-43 ...of TDP-43 N-terminal fragment (pdb:4bs2) containing two RRM domains (red helices, yellow beta ...
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Mice deficient in the C-terminal domain of TAR DNA-binding protein 43 develop age-dependent motor dysfunction associated with impaired Notch1−Akt signaling pathway
... in TDP-ΔC mice, we performed a microarray analysis using RNAs isolated from the aged TDP-ΔC mouse spinal ...by TDP-43 (Additional file 4: Table S3) according to the data on RNA targets of ...
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Interdomain interactions of the transactive response DNA binding protein 43 kDa (TDP-43)
... (18). TDP-43 also binds with HDAC6 mRNA and is necessary for maintaining HDAC6 mRNA and protein levels in vivo ...of TDP-43 decreased the amount of HDAC6 in HEK293 and SH-SY5Y cells and in Drosophila ...of ...
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