Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
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Mitochondrial and Ubiquitin Proteasome System Dysfunction in Ageing and Disease: Two Sides of the Same Coin?State of play in amyotrophic lateral sclerosis geneticsMitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?Oxidative stress and mitochondrial damage: importance in non-SOD1 ALSRare Manifestation of a c.290 C>T, p.Gly97Glu VCP MutationACTH (Acthar Gel) Reduces Toxic SOD1 Protein Linked to Amyotrophic Lateral Sclerosis in Transgenic Mice: A Novel ObservationThe valosin-containing protein is a novel mediator of mitochondrial respiration and cell survival in the heart in vivo.Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumPathogenic p62/SQSTM1 mutations impair energy metabolism through limitation of mitochondrial substrates.Mutations in valosin-containing protein (VCP) decrease ADP/ATP translocation across the mitochondrial membrane and impair energy metabolism in human neurons.Myogenic differentiation of VCP disease-induced pluripotent stem cells: A novel platform for drug discoveryProgressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALSα-Synuclein binds to the ER-mitochondria tethering protein VAPB to disrupt Ca2+ homeostasis and mitochondrial ATP productionThe ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solutionBrain temperature and its fundamental properties: a review for clinical neuroscientists.Genetic heterogeneity of amyotrophic lateral sclerosis: implications for clinical practice and research.GNX-4728, a novel small molecule drug inhibitor of mitochondrial permeability transition, is therapeutic in a mouse model of amyotrophic lateral sclerosis.A network of RNA and protein interactions in Fronto Temporal Dementia.The essential and downstream common proteins of amyotrophic lateral sclerosis: A protein-protein interaction network analysis.Doa1 targets ubiquitinated substrates for mitochondria-associated degradation.Enhanced uncoupling of the mitochondrial respiratory chain as a potential source for amyotrophic lateral sclerosisPathogenic Mutations in the Valosin-containing Protein/p97(VCP) N-domain Inhibit the SUMOylation of VCP and Lead to Impaired Stress Response.Drosophila clueless is involved in Parkin-dependent mitophagy by promoting VCP-mediated Marf degradation.Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants.The ER mitochondria calcium cycle and ER stress response as therapeutic targets in amyotrophic lateral sclerosis.Pathogenesis/genetics of frontotemporal dementia and how it relates to ALS.Targeting mitochondrial metal dyshomeostasis for the treatment of neurodegeneration.Systemic Analysis of miRNAs in PD Stress Condition: miR-5701 Modulates Mitochondrial-Lysosomal Cross Talk to Regulate Neuronal Death.Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts.Common and Divergent Mechanisms in Developmental Neuronal Remodeling and Dying Back Neurodegeneration.ER-mitochondria associations are regulated by the VAPB-PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43.Molecular Genetics of Neurodegenerative Dementias.New routes in frontotemporal dementia drug discovery.ALS/FTD-associated FUS activates GSK-3β to disrupt the VAPB-PTPIP51 interaction and ER-mitochondria associations.A computational model of motor neuron degeneration.Different dynamic movements of wild-type and pathogenic VCPs and their cofactors to damaged mitochondria in a Parkin-mediated mitochondrial quality control system.Pharmacological potential of Bidens pilosa L. and determination of bioactive compounds using UHPLC-QqQLIT-MS/MS and GC/MS.VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease.Mitochondrial Bioenergetics Is Altered in Fibroblasts from Patients with Sporadic Alzheimer's Disease.
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P2860
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
description
2013 nî lūn-bûn
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2013 թուականի Ապրիլին հրատարակուած գիտական յօդուած
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2013 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
2013年の論文
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2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
@zh-mo
2013年論文
@zh-tw
2013年论文
@wuu
name
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@ast
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@en
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@en-gb
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@nl
type
label
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@ast
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@en
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@en-gb
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@nl
prefLabel
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@ast
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@en
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@en-gb
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@nl
P2093
P2860
P50
P921
P3181
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P1476
Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels
@en
P2093
Antonio Canosa
Colin J Mahoney
Elisavet Preza
Hsiu-Chuan Wu
Richard W Orrell
Victoria S Burchell
P2860
P3181
P356
10.1016/J.NEURON.2013.02.028
P407
P50
P577
2013-04-10T00:00:00Z