Cargando…

Molecular Mechanisms Underlying TDP-43 Pathology in Cellular and Animal Models of ALS and FTLD

Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are neurodegenerative disorders that exist on a disease spectrum due to pathological, clinical and genetic overlap. In up to 97% of ALS cases and ~50% of FTLD cases, the primary pathological protein observed in affected...

Descripción completa

Detalles Bibliográficos
Autores principales:	Wood, Alistair, Gurfinkel, Yuval, Polain, Nicole, Lamont, Wesley, Lyn Rea, Sarah
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2021
Materias:	Review
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8125728/ https://www.ncbi.nlm.nih.gov/pubmed/33946763 http://dx.doi.org/10.3390/ijms22094705

Ejemplares similares

Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD–ALS spectrum disorders
por: Janssens, Jonathan, et al.
Publicado: (2013)

Towards a TDP-43-Based Biomarker for ALS and FTLD
por: Feneberg, Emily, et al.
Publicado: (2018)

The Pathobiology of TDP-43 C-Terminal Fragments in ALS and FTLD
por: Berning, Britt A., et al.
Publicado: (2019)

NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility
por: Gleixner, Amanda M., et al.
Publicado: (2022)

Evidence of cerebellar TDP-43 loss of function in FTLD-TDP
por: Pickles, Sarah, et al.
Publicado: (2022)

Therapeutic effect of berberine on TDP-43-related pathogenesis in FTLD and ALS
por: Chang, Cheng-Fu, et al.
Publicado: (2016)

Molecular Dissection of TDP-43 as a Leading Cause of ALS/FTLD
por: Tamaki, Yoshitaka, et al.
Publicado: (2022)

Classification of FTLD-TDP cases into pathological subtypes using antibodies against phosphorylated and non-phosphorylated TDP43
por: Tan, Rachel H, et al.
Publicado: (2013)

Codon-optimized TDP-43 mediates neurodegeneration in a Drosophila model of ALS/FTLD
por: Yusuff, Tanzeen, et al.
Publicado: (2023)

TDP-43 Vasculopathy in the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis (sALS) and Frontal Cortex in sALS/FTLD-TDP
por: Ferrer, Isidro, et al.
Publicado: (2021)

Antibody against TDP-43 phosphorylated at serine 375 suggests conformational differences of TDP-43 aggregates among FTLD–TDP subtypes
por: Neumann, Manuela, et al.
Publicado: (2020)

Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
por: Fiondella, Luigi, et al.
Publicado: (2023)

TDP-43 forms amyloid filaments with a distinct fold in type A FTLD-TDP
por: Arseni, Diana, et al.
Publicado: (2023)

Diffuse argyrophilic grain disease with TDP-43 proteinopathy and neuronal intermediate filament inclusion disease: FTLD with mixed tau, TDP-43 and FUS pathologies
por: Koga, Shunsuke, et al.
Publicado: (2023)

mTh1 driven expression of hTDP-43 results in typical ALS/FTLD neuropathological symptoms
por: Scherz, Barbara, et al.
Publicado: (2018)

Publisher Correction to: Diffuse argyrophilic grain disease with TDP-43 proteinopathy and neuronal intermediate filament inclusion disease: FTLD with mixed tau, TDP-43 and FUS pathologies
por: Koga, Shunsuke, et al.
Publicado: (2023)

Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U
por: Tsai, Kuen-Jer, et al.
Publicado: (2010)

Neuronal VCP loss of function recapitulates FTLD-TDP pathology
por: Wani, Abubakar, et al.
Publicado: (2021)

Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLD
por: Diaper, Danielle C., et al.
Publicado: (2013)

Metals in ALS TDP-43 Pathology
por: Koski, Lassi, et al.
Publicado: (2021)

C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation
por: Josephs, Keith A., et al.
Publicado: (2019)

Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD
por: Khosravi, Bahram, et al.
Publicado: (2017)

Lipid alterations in human frontal cortex in ALS‐FTLD‐TDP43 proteinopathy spectrum are partly related to peroxisome impairment
por: Andrés‐Benito, Pol, et al.
Publicado: (2021)

Shared brain transcriptomic signature in TDP-43 type A FTLD patients with or without GRN mutations
por: Pottier, Cyril, et al.
Publicado: (2021)

The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons()
por: Honda, Daiyu, et al.
Publicado: (2013)

Pathogenic Mutation of TDP-43 Impairs RNA Processing in a Cell Type-Specific Manner: Implications for the Pathogenesis of ALS/FTLD
por: Imaizumi, Kent, et al.
Publicado: (2022)

Regional and hemispheric susceptibility of the temporal lobe to FTLD-TDP type C pathology
por: Borghesani, V., et al.
Publicado: (2020)

Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD
por: Ward, Michael E., et al.
Publicado: (2014)

Neuronal overexpression of hTDP-43 in Caenorhabditis elegans mimics the cellular pathology commonly observed in TDP-43 proteinopathies
por: Koopman, Mandy, et al.
Publicado: (2023)

The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients
por: Collins, Mahlon, et al.
Publicado: (2012)

Spreading of TDP-43 pathology via pyramidal tract induces ALS-like phenotypes in TDP-43 transgenic mice
por: Ding, Xuebing, et al.
Publicado: (2021)

TDP-43 Pathology in Alzheimer’s Disease
por: Meneses, Axel, et al.
Publicado: (2021)

Aggregation-prone TDP-43 sequesters and drives pathological transitions of free nuclear TDP-43
por: Keating, Sean S., et al.
Publicado: (2023)

Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS
por: Mitchell, Jacqueline C, et al.
Publicado: (2015)

A C-Terminally Truncated TDP-43 Splice Isoform Exhibits Neuronal Specific Cytoplasmic Aggregation and Contributes to TDP-43 Pathology in ALS
por: Shenouda, Marc, et al.
Publicado: (2022)

The Pathological Phenotypes of Human TDP-43 Transgenic Mouse Models Are Independent of Downregulation of Mouse Tdp-43
por: Xu, Ya-Fei, et al.
Publicado: (2013)

Monomerization of TDP-43 is a key determinant for inducing TDP-43 pathology in amyotrophic lateral sclerosis
por: Oiwa, Kotaro, et al.
Publicado: (2023)

Which TDP-43 aggregates are toxic in ALS?
por: Valle, Cristiana, et al.
Publicado: (2016)

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
por: Rusina, Robert, et al.
Publicado: (2011)

Detection and quantification of novel C‐terminal TDP‐43 fragments in ALS‐TDP
por: Feneberg, Emily, et al.
Publicado: (2021)

Cannot write session to /tmp/vufind_sessions/sess_njfm3u92c6o3cis6fiobtpabsk