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ALS & FTD: TDP-43

ALS & FTD: TDP-43 diagram by Atthapon Raksthaput, Shutterstock
Atthapon Raksthaput, Shutterstock

ALS shares with a considerable proportion of FTD cases the same neuropathological substrate, namely, inclusions of abnormally phosphorylated protein tdp-43 (ptdp-43). Here are the latest discoveries pertaining to ptdp-43 and these diseases.

Top 20 most recent papers
bioRxiv

ATN status in amnestic and non-amnestic Alzheimer’s disease and frontotemporal lobar degeneration

bioRxivDecember 19, 2019
Katheryn A.Q. CousinsJeffrey S. Phillips
5
Science Translational Medicine

Activity of the poly(A) binding protein MSUT2 determines susceptibility to pathological tau in the mammalian brain

Science Translational MedicineDecember 20, 2019
Jeanna M WheelerBrian C Kraemer
54
12
2
bioRxiv

TDP-43 dysfunction restricts dendritic complexity by inhibiting CREB activation and altering gene expression

bioRxivDecember 13, 2019
Josiah J. HerzogSuzanne Paradis
2
Alzheimer's & Dementia : the Journal of the Alzheimer's Association

Utility of the global CDR® plus NACC FTLD rating and development of scoring rules: Data from the ARTFL/LEFFTDS Consortium

Alzheimer's & Dementia : the Journal of the Alzheimer's AssociationJanuary 9, 2020
Toji MiyagawaZbigniew Wszolek
1
1
bioRxiv

A non-invasive digital biomarker for the detection of rest disturbances in the SOD1G93A mouse model of ALS

bioRxivDecember 27, 2019
Elisabetta GoliniSilvia Mandillo
2
Scientific Reports

SMN complex member Gemin3 self-interacts and has a functional relationship with ALS-linked proteins TDP-43, FUS and Sod1

Scientific ReportsDecember 12, 2019
Rebecca CacciottoloRuben J Cauchi
3
1
1
The Journal of Applied Laboratory Medicine

Detection of Alzheimer Disease Pathology in Patients Using Biochemical Biomarkers: Prospects and Challenges for Use in Clinical Practice

The Journal of Applied Laboratory MedicineDecember 19, 2019
Leslie M ShawJohn Q Trojanowski
1
17
bioRxiv

Redox-mediated regulation of a labile, evolutionarily conserved cross-β structure formed by the TDP43 low complexity domain

bioRxivDecember 27, 2019
Yi LinSteven L. McKnight
7
Brain : a Journal of Neurology

Homozygous GRN mutations: new phenotypes and new insights into pathological and molecular mechanisms

Brain : a Journal of NeurologyDecember 20, 2019
Vincent HuinIsabelle Le Ber
15
1
Alzheimer's & Dementia : the Journal of the Alzheimer's Association

Genetic screening of a large series of North American sporadic and familial frontotemporal dementia cases

Alzheimer's & Dementia : the Journal of the Alzheimer's AssociationJanuary 9, 2020
Eliana Marisa RamosGiovanni Coppola
1
1
Alzheimer's & Dementia : the Journal of the Alzheimer's Association

Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort

Alzheimer's & Dementia : the Journal of the Alzheimer's AssociationJanuary 9, 2020
Hilary W HeuerARTFL and LEFFTDS consortia
4
1
Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration

The first case of the TARDBP p.G294V mutation in a homozygous state: is a single pathogenic allele sufficient to cause ALS?

Amyotrophic Lateral Sclerosis & Frontotemporal DegenerationDecember 20, 2019
Lucia CorradoSandra D'Alfonso
Alzheimer's & Dementia : the Journal of the Alzheimer's Association

Assessment of executive function declines in presymptomatic and mildly symptomatic familial frontotemporal dementia: NIH-EXAMINER as a potential clinical trial endpoint

Alzheimer's & Dementia : the Journal of the Alzheimer's AssociationJanuary 9, 2020
Adam M StaffaroniARTFL/LEFFTDS Consortium
Alzheimer's & Dementia : Diagnosis, Assessment & Disease Monitoring

Nonlinear Z-score modeling for improved detection of cognitive abnormality

Alzheimer's & Dementia : Diagnosis, Assessment & Disease MonitoringDecember 25, 2019
John KornakARTFL/LEFFTDS Consortium
2
Revue neurologique

Pre-symptomatic diagnosis in ALS

Revue neurologiqueJanuary 15, 2020
P Corciaon Behalf the FILSLAN network
Neuron

Antibody Therapy Targeting RAN Proteins Rescues C9 ALS/FTD Phenotypes in C9orf72 Mouse Model

NeuronDecember 14, 2019
Lien NguyenLaura P W Ranum
19
2
Neuron

Glucocerebrosidase Activity Modulates Neuronal Susceptibility to Pathological α-Synuclein Insult

NeuronJanuary 4, 2020
Michael X HendersonVirginia M Y Lee
40
1
Neurobiology of Disease

Dysregulation of Rac or Rho elicits death of motor neurons and activation of these GTPases is altered in the G93A mutant hSOD1 mouse model of amyotrophic lateral sclerosis

Neurobiology of DiseaseJanuary 14, 2020
Trisha R StankiewiczDaniel A Linseman
EMBO Molecular Medicine

Active poly-GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model

EMBO Molecular MedicineDecember 21, 2019
Qihui ZhouDieter Edbauer
32
1
Neurobiology of Disease

Characterization of novel conformation-selective α-synuclein antibodies as potential immunotherapeutic agents for Parkinson's disease

Neurobiology of DiseaseDecember 15, 2019
Michael X HendersonKelvin C Luk
15
1

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