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ProteomicsVCP loss of function in neurons recapitulates FTLD-TDP pathology
Dominant mutations in unrelated genes cause fronto-temporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) and include VCP, which is associated with multisystem proteinopathy (MSP). Conditional inactivation of VCP in postnatal forebrain neurons (VCP cKO) caused cortical brain atrophy, neurona...
ORGANISM(S): Mus musculus (Mouse) 
2021-08-06 | PXD026685 | Pride
Transcriptomic Vcp/p97 Proteomic Fronto-temporal lobar degeneration 
UnknownImpact of long- and short-range fibre depletion on the cognitive deficits of fronto-temporal dementia.
Not available
S-EPMC8824472 | biostudies-literature
UnknownLysosome size, motility and stress response regulated by fronto-temporal dementia modifier TMEM106B.
Not available
S-EPMC4145808 | biostudies-literature
UnknownClusters of co-abundant proteins in the brain cortex associated with fronto-temporal lobar degeneration.
Not available
S-EPMC10035199 | biostudies-literature
UnknownPresymptomatic and early pathological features of MAPT-associated frontotemporal lobar degeneration.
Not available
S-EPMC10394953 | biostudies-literature
UnknownFace shape and face identity processing in behavioral variant fronto-temporal dementia: A specific deficit for familiarity and name recognition of famous faces.
Not available
S-EPMC4893012 | biostudies-literature
TranscriptomicsComparative gene expression profiling of Drosophila primary neuronal cultures expressing or not the human Tau0N4RWT protein
Deciphering the pathophysiological mechanisms that lead from the alteration of human Tau biology to neuronal death in tauopathies including Alzheimer's disease (AD), fronto-temporal dementia with parkinsonism linked to chromosome 17 (FTDP-17), Pick's disease (PiD), corticobasal degeneration (CBD) an...
ORGANISM(S): Drosophila melanogaster 
2020-02-11 | E-MTAB-8712 | biostudies-arrayexpress
TranscriptomicsNeuronal VCP loss of function recapitulates FTLD-TDP pathology
The pathogenic mechanism by which dominant mutations in VCP cause multisystem proteinopathy (MSP), a rare neurodegenerative disease that presents as fronto-temporal lobar degeneration with TDP-43 inclusions (FTLD-TDP), remains unclear. To explore this, we inactivated VCP in murine postnatal forebrai...
ORGANISM(S): Mus musculus 
2021-06-16 | GSE178257 | GEO
UnknownFronto-temporal dementia risk gene TMEM106B has opposing effects in different lysosomal storage disorders.
Not available
S-EPMC7990118 | biostudies-literature
GenomicsEWS and FUS Bind a Subset of Transcribed Genes Encoding Proteins Enriched in RNA Regulatory Functions
Background FUS (TLS) and EWS (EWSR1) belong to the FET-protein family of RNA and DNA binding proteins. FUS and EWS are structurally and functionally related and participate in transcriptional regulation and RNA splicing. FUS and EWS are identified in translocation generated cancer fusion proteins an...
ORGANISM(S): Homo sapiens 
2015-11-19 | GSE73492 | GEO
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