Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait.
about
The linkage of Kennedy's neuron disease to ARA24, the first identified androgen receptor polyglutamine region-associated coactivatorAndrogen-modulating agents for spinal bulbar muscular atrophy/Kennedy's diseaseMimics and chameleons in motor neurone diseaseAndrogen-dependent pathology demonstrates myopathic contribution to the Kennedy disease phenotype in a mouse knock-in modelTandem repeat copy-number variation in protein-coding regions of human genes.Pathogenic mechanisms and therapeutic strategies in spinobulbar muscular atrophyThe unstable repeats--three evolving faces of neurological diseasePolyglutamine neurodegeneration: expanded glutamines enhance native functionsThe polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex.Genotype-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.Protein arginine methyltransferase 6 enhances polyglutamine-expanded androgen receptor function and toxicity in spinal and bulbar muscular atrophy.Small ubiquitin-like modifier (SUMO) modification of the androgen receptor attenuates polyglutamine-mediated aggregationDisrupting SUMOylation enhances transcriptional function and ameliorates polyglutamine androgen receptor-mediated disease.Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy.Overexpression of wild-type androgen receptor in muscle recapitulates polyglutamine disease.Androgen regulation of axon growth and neurite extension in motoneuronsPrenatal flutamide enhances survival in a myogenic mouse model of spinal bulbar muscular atrophySpinal and bulbar muscular atrophy: pathogenesis and clinical managementSensory involvement in spinal-bulbar muscular atrophy (Kennedy's disease).Increased mitophagy in the skeletal muscle of spinal and bulbar muscular atrophy patientsX-linked bulbo-spinal neuronopathy: a family study of three patients.Comparison of sporadic and familial disease amongst 580 cases of motor neuron disease.Disruption of nongenomic testosterone signaling in a model of spinal and bulbar muscular atrophy.Global gene expression profiling of somatic motor neuron populations with different vulnerability identify molecules and pathways of degeneration and protectionAndrogen receptor mutation in Kennedy's diseaseMicroarray analysis of gene expression by skeletal muscle of three mouse models of Kennedy disease/spinal bulbar muscular atrophyFamilial bulbospinal neuronopathy with optic atrophy: a distinct entityAntiandrogen flutamide protects male mice from androgen-dependent toxicity in three models of spinal bulbar muscular atrophy.Distinguishing clinical and electrodiagnostic features of X-linked bulbospinal neuronopathy.Correlation of clinical and molecular features in spinal bulbar muscular atrophy.Animal models of Kennedy disease.Androgen receptor and Kennedy disease/spinal bulbar muscular atrophyTriplet repeat expansion in neuromuscular disease.Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice.The use of sugammadex in a patient with Kennedy's disease under general anesthesiaPharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease.Tissue-specific somatic mosaicism in spinal and bulbar muscular atrophy is dependent on CAG-repeat length and androgen receptor--gene expression level.Therapeutic approaches to delay the onset of Alzheimer's diseaseA rapidly progressive autosomal dominant scapulohumeral form of spinal muscular atrophy.
P2860
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P2860
Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait.
description
1968 nî lūn-bûn
@nan
1968 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
1968 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
1968年の論文
@ja
1968年論文
@yue
1968年論文
@zh-hant
1968年論文
@zh-hk
1968年論文
@zh-mo
1968年論文
@zh-tw
1968年论文
@wuu
name
Progressive proximal spinal an ...... A sex-linked recessive trait.
@ast
Progressive proximal spinal an ...... A sex-linked recessive trait.
@en
Progressive proximal spinal an ...... A sex-linked recessive trait.
@nl
type
label
Progressive proximal spinal an ...... A sex-linked recessive trait.
@ast
Progressive proximal spinal an ...... A sex-linked recessive trait.
@en
Progressive proximal spinal an ...... A sex-linked recessive trait.
@nl
prefLabel
Progressive proximal spinal an ...... A sex-linked recessive trait.
@ast
Progressive proximal spinal an ...... A sex-linked recessive trait.
@en
Progressive proximal spinal an ...... A sex-linked recessive trait.
@nl
P2093
P356
P1433
P1476
Progressive proximal spinal an ...... A sex-linked recessive trait.
@en
P2093
P304
P356
10.1212/WNL.18.7.671
P407
P577
1968-07-01T00:00:00Z