Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS - Wikidata - awgldk - TriplyDB

Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS

Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS

http://www.wikidata.org/entity/Q34139970

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VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis Mitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?Oxidative stress and mitochondrial damage: importance in non-SOD1 ALS The omega-3 fatty acid eicosapentaenoic acid accelerates disease progression in a model of amyotrophic lateral sclerosis Pyrimethamine decreases levels of SOD1 in leukocytes and cerebrospinal fluid of ALS patients: a phase I pilot study Mitochondrial dysfunction in neurodegenerative diseases Exploring new pathways of neurodegeneration in ALS: the role of mitochondria quality control Morphological and bioenergetic demands underlying the mitophagy in post-mitotic neurons: the pink-parkin pathway Mitochondrial lipids in neurodegeneration Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis Intermolecular transmission of superoxide dismutase 1 misfolding in living cells.Palmitoylation of superoxide dismutase 1 (SOD1) is increased for familial amyotrophic lateral sclerosis-linked SOD1 mutants Acyl coenzyme A thioesterase 7 regulates neuronal fatty acid metabolism to prevent neurotoxicity.Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS Gene expression profiling for human iPS-derived motor neurons from sporadic ALS patients reveals a strong association between mitochondrial functions and neurodegeneration.SOD1, an unexpected novel target for cancer therapy New Therapeutics to Modulate Mitochondrial Function in Neurodegenerative Disorders.Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset.Oxidative stress, mitochondrial damage and neurodegenerative diseases Direct observation of defects and increased ion permeability of a membrane induced by structurally disordered Cu/Zn-superoxide dismutase aggregates.Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity.ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import.Mechanisms, models and biomarkers in amyotrophic lateral sclerosis Perturbations in intracellular Ca2+ handling in skeletal muscle in the G93A*SOD1 mouse model of amyotrophic lateral sclerosis.Repetitive nerve stimulation transiently opens the mitochondrial permeability transition pore in motor nerve terminals of symptomatic mutant SOD1 mice Bee venom effects on ubiquitin proteasome system in hSOD1(G85R)-expressing NSC34 motor neuron cells Increased axonal mitochondrial mobility does not slow amyotrophic lateral sclerosis (ALS)-like disease in mutant SOD1 mice.Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability The role of mitochondrial DNA mutation on neurodegenerative diseases.Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1.Activation of the endoplasmic reticulum stress response in skeletal muscle of G93A*SOD1 amyotrophic lateral sclerosis mice.In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space Inhibition of Cytohesins Protects against Genetic Models of Motor Neuron Disease.Modeling mitochondrial dysfunctions in the brain: from mice to men.Mitochondrial dynamic abnormalities in amyotrophic lateral sclerosis.Effects of Cellular Pathway Disturbances on Misfolded Superoxide Dismutase-1 in Fibroblasts Derived from ALS Patients Mutant SOD1 forms ion channel: implications for ALS pathophysiology.The mitochondrial calcium regulator cyclophilin D is an essential component of oestrogen-mediated neuroprotection in amyotrophic lateral sclerosis

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P2860

Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS

http://www.wikidata.org/entity/Q34139970

description

2010 nî lūn-bûn

@nan

2010 թուականի Օգոստոսին հրատարակուած գիտական յօդուած

@hyw

2010 թվականի օգոստոսին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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name

Misfolded mutant SOD1 directly ...... a mouse model of inherited ALS

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Misfolded mutant SOD1 directly ...... a mouse model of inherited ALS

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Misfolded mutant SOD1 directly ...... a mouse model of inherited ALS

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Misfolded mutant SOD1 directly ...... a mouse model of inherited ALS

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Misfolded mutant SOD1 directly ...... a mouse model of inherited ALS

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Hristelina Ilieva

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Nir Arbel

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Sandrine Da Cruz

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P2860

Bcl-2 family proteins regulate the release of apoptogenic cytochrome c by the mitochondrial channel VDAC

PINK1/Parkin-mediated mitophagy is dependent on VDAC1 and p62/SQSTM1

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis

SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model

ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS

The voltage-dependent anion channel: an essential player in apoptosis

Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models

Permeation of hydrophilic solutes through mitochondrial outer membranes: review on mitochondrial porins

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4

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Adrian Israelson

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46008797

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amyotrophic lateral sclerosis

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2941987

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