GABAergic, GDNF and Sphingolipid Pathways Are Potentially Druggable Targets in Neurodegenerative Diseases: Lumping Is Better than Splitting
Sign Up to like & getrecommendations! Published in 2022 at "Movement Disorders Clinical Practice"
DOI: 10.1002/mdc3.13648
Abstract: The clinical disorders caused by frontotemporal lobar degeneration pathologies (FTLD) are highly heterogeneous in their pathology and phenotypes. Patients are typically diagnosed as having one of several clinical entities de fi ned by speci fi… read more here.
Keywords: ftld tdp; sphingolipid pathways; pathology; gdnf sphingolipid ... See more keywords
Genome-wide analyses as part of the international FTLD-TDP whole-genome sequencing consortium reveals novel disease risk factors and increases support for immune dysfunction in FTLD
Sign Up to like & getrecommendations! Published in 2019 at "Acta Neuropathologica"
DOI: 10.1007/s00401-019-01962-9
Abstract: Frontotemporal lobar degeneration with neuronal inclusions of the TAR DNA-binding protein 43 (FTLD-TDP) represents the most common pathological subtype of FTLD. We established the international FTLD-TDP whole-genome sequencing consortium to thoroughly characterize the known genetic… read more here.
Keywords: ftld; ftld tdp; whole genome; tdp patients ... 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
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
Degeneration of the locus coeruleus is a common feature of tauopathies and distinct from TDP-43 proteinopathies in the frontotemporal lobar degeneration spectrum
Sign Up to like & getrecommendations! Published in 2020 at "Acta Neuropathologica"
DOI: 10.1007/s00401-020-02210-1
Abstract: Neurodegeneration of the locus coeruleus (LC) in age-related neurodegenerative diseases such as Alzheimer’s disease (AD) is well documented. However, detailed studies of LC neurodegeneration in the full spectrum of frontotemporal lobar degeneration (FTLD) proteinopathies comparing… read more here.
Keywords: ftld; ftld tau; neurodegeneration; ftld tdp ... See more keywords
Amyloid fibrils in disease FTLD-TDP are composed of TMEM106B not TDP-43.
Sign Up to like & getrecommendations! Published in 2022 at "Nature"
DOI: 10.1038/s41586-022-04670-9
Abstract: Frontotemporal lobar degeneration (FTLD) is the third most common neurodegenerative condition, following only Alzheimer's and Parkinson's diseases1. FTLD typically presents in 45-64-year-olds with behavioral changes or progressive decline of language skills2. The subtype FTLD-TDP is… read more here.
Keywords: ftld tdp; tdp; disease; amyloid fibrils ... See more keywords
FTLD-TDP With and Without GRN Mutations Cause Different Patterns of CA1 Pathology.
Sign Up to like & getrecommendations! Published in 2019 at "Journal of neuropathology and experimental neurology"
DOI: 10.1093/jnen/nlz059
Abstract: Heterozygous loss-of-function mutations in the GRN gene lead to progranulin (PGRN) haploinsufficiency and cause frontotemporal lobar degeneration with TDP-43 pathology type A (FTLD-TDP type A). PGRN is a highly conserved, secreted glycoprotein and functions in… read more here.
Keywords: grn mutations; ftld tdp; type; pathology ... See more keywords
Distinguishing post‐translational modifications in dominantly inherited frontotemporal dementias: FTLD‐TDP Type A (GRN) vs Type B (C9orf72)
Sign Up to like & getrecommendations! Published in 2022 at "Neuropathology and Applied Neurobiology"
DOI: 10.1111/nan.12836
Abstract: Frontotemporal dementias are neuropathologically characterised by frontotemporal lobar degeneration (FTLD). Intraneuronal inclusions of transactive response DNA‐binding protein 43 kDa (TDP‐43) are the defining pathological hallmark of approximately half of the FTLD cases, being referred to as… read more here.
Keywords: translational modifications; post translational; ftld tdp; frontotemporal dementias ... See more keywords
C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation
Sign Up to like & getrecommendations! Published in 2019 at "Acta Neuropathologica Communications"
DOI: 10.1186/s40478-019-0755-x
Abstract: The transactive response DNA binding protein of 43 kDa (TDP-43) is an intranuclear protein involved in RNA splicing. Abnormally deposited TDP-43 is found in the brains of patients with frontotemporal lobar degeneration (FTLD). Different morphological… read more here.
Keywords: full length; tdp; ftld tdp; burden ... See more keywords
Empiric Methods to Account for Pre-analytical Variability in Digital Histopathology in Frontotemporal Lobar Degeneration
Sign Up to like & getrecommendations! Published in 2019 at "Frontiers in Neuroscience"
DOI: 10.3389/fnins.2019.00682
Abstract: Digital pathology is increasingly prominent in neurodegenerative disease research, but variability in immunohistochemical staining intensity between staining batches prevents large-scale comparative studies. Here we provide a statistically rigorous method to account for staining batch effects… read more here.
Keywords: ftld; ftld tau; histopathology; pathology ... See more keywords