Mitochondrial abnormalities in spinal and bulbar muscular atrophy
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Idebenone for the treatment of Duchenne muscular dystrophyIdebenone for the treatment of Duchenne muscular dystrophyMouse models of polyglutamine diseases: review and data table. Part IPathogenic mechanisms and therapeutic strategies in spinobulbar muscular atrophyDeveloping treatment for spinal and bulbar muscular atrophyThe 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 atrophyGenotype-phenotype correlation in Chinese patients with spinal and bulbar muscular atrophyDecreased 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 INQO1-dependent redox cycling of idebenone: effects on cellular redox potential and energy levelsMitochondria Initiate and Regulate SarcopeniaPeripheral 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 managementIncreased mitophagy in the skeletal muscle of spinal and bulbar muscular atrophy patientsTherapeutic approaches to spinal and bulbar muscular atrophyMicroarray analysis of gene expression by skeletal muscle of three mouse models of Kennedy disease/spinal bulbar muscular atrophyMacroautophagy 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 terminalsCan the vector space model be used to identify biological entity activities?Stem cell-derived motor neurons from spinal and bulbar muscular atrophy patientsPolyglutamine-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 repeatPathogenesis 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 sclerosisActivation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradationMechanisms 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 dietClearance 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
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
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
@en
Mitochondrial abnormalities in spinal and bulbar muscular atrophy.
@nl
P2093
P2860
P356
P1476
Mitochondrial abnormalities in spinal and bulbar muscular atrophy
@en
P2093
Barrington G Burnett
George G Harmison
Kenneth H Fischbeck
Kristin Meyertholen
Maria Pennuto
Srikanth Ranganathan
P2860
P356
10.1093/HMG/DDN310
P577
2008-09-29T00:00:00Z