Mitochondrial abnormalities in spinal and bulbar muscular atrophy - Wikidata - awgldk - TriplyDB

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

http://www.wikidata.org/entity/Q37102121

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Idebenone for the treatment of Duchenne muscular dystrophy Idebenone for the treatment of Duchenne muscular dystrophy Mouse models of polyglutamine diseases: review and data table. Part I Pathogenic mechanisms and therapeutic strategies in spinobulbar muscular atrophy Developing treatment for spinal and bulbar muscular atrophy The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex.A small-molecule Nrf1 and Nrf2 activator mitigates polyglutamine toxicity in spinal and bulbar muscular atrophy Genotype-phenotype correlation in Chinese patients with spinal and bulbar muscular atrophy Decreased Peak Expiratory Flow Associated with Muscle Fiber-Type Switching in Spinal and Bulbar Muscular Atrophy.Features of idebenone and related short-chain quinones that rescue ATP levels under conditions of impaired mitochondrial complex I NQO1-dependent redox cycling of idebenone: effects on cellular redox potential and energy levels Mitochondria Initiate and Regulate Sarcopenia Peripheral androgen receptor gene suppression rescues disease in mouse models of spinal and bulbar muscular atrophy.Disrupting SUMOylation enhances transcriptional function and ameliorates polyglutamine androgen receptor-mediated disease.Spinal and bulbar muscular atrophy: pathogenesis and clinical management Increased mitophagy in the skeletal muscle of spinal and bulbar muscular atrophy patients Therapeutic approaches to spinal and bulbar muscular atrophy Microarray analysis of gene expression by skeletal muscle of three mouse models of Kennedy disease/spinal bulbar muscular atrophy Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice.SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals Can the vector space model be used to identify biological entity activities?Stem cell-derived motor neurons from spinal and bulbar muscular atrophy patients Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA.Activation of the stress proteome as a mechanism for small molecule therapeutics.SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse.Spinal and Bulbar Muscular Atrophy.Early onset and novel features in a spinal and bulbar muscular atrophy patient with a 68 CAG repeat Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy (SBMA).Absence of disturbed axonal transport in spinal and bulbar muscular atrophy.Contractile dysfunction in muscle may underlie androgen-dependent motor dysfunction in spinal bulbar muscular atrophy.Impaired motoneuronal retrograde transport in two models of SBMA implicates two sites of androgen action.Mitochondrial dysfunction in Kennedy's disease: a new pharmacological target?Current status of treatment of spinal and bulbar muscular atrophy.Nemo-like kinase is a novel regulator of spinal and bulbar muscular atrophy.Serum caspase-9 levels are increased in patients with amyotrophic lateral sclerosis Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation Mechanisms mediating spinal and bulbar muscular atrophy: investigations into polyglutamine-expanded androgen receptor function and dysfunction.MiR-298 Counteracts Mutant Androgen Receptor Toxicity in Spinal and Bulbar Muscular Atrophy.Glycolytic-to-oxidative fiber-type switch and mTOR signaling activation are early-onset features of SBMA muscle modified by high-fat diet Clearance of the mutant androgen receptor in motoneuronal models of spinal and bulbar muscular atrophy.

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Mitochondrial abnormalities in spinal and bulbar muscular atrophy

http://www.wikidata.org/entity/Q37102121

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article científic

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article scientifique

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articolo scientifico

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artigo científico

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bilimsel makale

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scientific article published on 29 September 2008

@en

vedecký článok

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vetenskaplig artikel

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videnskabelig artikel

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vědecký článek

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name

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

@en

Mitochondrial abnormalities in spinal and bulbar muscular atrophy.

@nl

type

label

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

@en

Mitochondrial abnormalities in spinal and bulbar muscular atrophy.

@nl

prefLabel

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

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Mitochondrial abnormalities in spinal and bulbar muscular atrophy.

@nl

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Human Molecular Genetics

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Mitochondrial abnormalities in spinal and bulbar muscular atrophy

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Barrington G Burnett

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George G Harmison

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Kenneth H Fischbeck

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Kristin Meyertholen

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Maria Pennuto

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Srikanth Ranganathan

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P2860

14-3-3 inhibits Bad-induced cell death through interaction with serine-136

Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator

An endoplasmic reticulum stress-specific caspase cascade in apoptosis. Cytochrome c-independent activation of caspase-9 by caspase-12

Androgen-dependent pathology demonstrates myopathic contribution to the Kennedy disease phenotype in a mouse knock-in model

A new mathematical model for relative quantification in real-time RT-PCR

Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1

Cytochrome c oxidase subunit Vb interacts with human androgen receptor: a potential mechanism for neurotoxicity in spinobulbar muscular atrophy

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy.

Survival factor-mediated BAD phosphorylation raises the mitochondrial threshold for apoptosis

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10.1093/HMG/DDN310

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1

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