The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis.
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The genetics and neuropathology of frontotemporal lobar degenerationMutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALSRing of Change: CDC48/p97 Drives Protein Dynamics at ChromatinPhenotypic Heterogeneity of Monogenic Frontotemporal DementiaCardiac-Restricted Expression of VCP/TER94 RNAi or Disease Alleles Perturbs Drosophila Heart Structure and Impairs Function.The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathologyAltered Intersubunit Communication Is the Molecular Basis for Functional Defects of Pathogenic p97 MutantsThe ubiquitin-selective chaperone Cdc48/p97 associates with Ubx3 to modulate monoubiquitylation of histone H2B.Frontotemporal dementia: a bridge between dementia and neuromuscular diseaseImpact of air pollutants on oxidative stress in common autophagy-mediated aging diseasesRare Manifestation of a c.290 C>T, p.Gly97Glu VCP MutationControl of p97 function by cofactor bindingThe requirement for Cdc48/p97 in nuclear protein quality control degradation depends on the substrate and correlates with substrate insolubilityExploring new pathways of neurodegeneration in ALS: the role of mitochondria quality controlA Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem ProteinopathyThe Myoblast C2C12 Transfected with Mutant Valosin-Containing Protein Exhibits Delayed Stress Granule Resolution on Oxidative Stress.Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLDA Novel Protocol for Directed Differentiation of C9orf72-Associated Human Induced Pluripotent Stem Cells Into Contractile Skeletal Myotubes.Predicting functional decline and survival in amyotrophic lateral sclerosis.Involvement of peripheral and central nervous systems in a valosin-containing protein mutation.Myogenic differentiation of VCP disease-induced pluripotent stem cells: A novel platform for drug discoveryA Mighty "Protein Extractor" of the Cell: Structure and Function of the p97/CDC48 ATPase.Rare mutations in SQSTM1 modify susceptibility to frontotemporal lobar degenerationExercise training reverses skeletal muscle atrophy in an experimental model of VCP disease.A network of RNA and protein interactions in Fronto Temporal Dementia.CHMP5 controls bone turnover rates by dampening NF-κB activity in osteoclastsGlobal gene profiling of VCP-associated inclusion body myopathy.A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouseAsymmetric pathology in primary progressive aphasia with progranulin mutations and TDP inclusions.Novel mutation in VCP gene causes atypical amyotrophic lateral sclerosis.Structural and functional deviations in disease-associated p97 mutants.Genotype-phenotype studies of VCP-associated inclusion body myopathy with Paget disease of bone and/or frontotemporal dementiaStructure and function of the AAA+ ATPase p97/Cdc48p.Pathogenic Mutations in the Valosin-containing Protein/p97(VCP) N-domain Inhibit the SUMOylation of VCP and Lead to Impaired Stress Response.Phenotypic variability in three families with valosin-containing protein mutation.A review of quality of life after predictive testing for and earlier identification of neurodegenerative diseases.Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease."Get the Balance Right": Pathological Significance of Autophagy Perturbation in Neuromuscular DisordersWidespread RNA metabolism impairment in sporadic inclusion body myositis TDP43-proteinopathy.Cdc48: a swiss army knife of cell biology.
P2860
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P2860
The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis.
description
article científic
@ca
article scientifique
@fr
articol științific
@ro
articolo scientifico
@it
artigo científico
@gl
artigo científico
@pt
artigo científico
@pt-br
artikel ilmiah
@id
artikull shkencor
@sq
artículo científico
@es
name
The multiple faces of valosin- ...... amyotrophic lateral sclerosis.
@en
The multiple faces of valosin- ...... amyotrophic lateral sclerosis.
@nl
type
label
The multiple faces of valosin- ...... amyotrophic lateral sclerosis.
@en
The multiple faces of valosin- ...... amyotrophic lateral sclerosis.
@nl
prefLabel
The multiple faces of valosin- ...... amyotrophic lateral sclerosis.
@en
The multiple faces of valosin- ...... amyotrophic lateral sclerosis.
@nl
P2093
P2860
P1476
The multiple faces of valosin- ...... amyotrophic lateral sclerosis
@en
P2093
Angèle Nalbandian
Barbara Martin
Charles Smith
Christopher Nguyen
Giles D Watts
Jouni Vesa
Mallikarjun Badadani
Prachi Rana
Sandra Donkervoort
P2860
P2888
P304
P356
10.1007/S12031-011-9627-Y
P577
2011-09-03T00:00:00Z