Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy.
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Exendin-4 improves glycemic control, ameliorates brain and pancreatic pathologies, and extends survival in a mouse model of Huntington's diseaseHDAC4-myogenin axis as an important marker of HD-related skeletal muscle atrophyThe chicken or the egg: mitochondrial dysfunction as a cause or consequence of toxicity in Huntington's diseaseHuntington's diseaseShaping the role of mitochondria in the pathogenesis of Huntington's diseaseRole of oxidative DNA damage in mitochondrial dysfunction and Huntington's disease pathogenesisEnergy failure: does it contribute to neurodegeneration?Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolismMutant huntingtin impairs immune cell migration in Huntington disease.Early energy deficit in Huntington disease: identification of a plasma biomarker traceable during disease progression.Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease.High-resolution respirometry of fine-needle muscle biopsies in pre-manifest Huntington's disease expansion mutation carriers shows normal mitochondrial respiratory function.Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation.Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease.Skeletal muscle pathology in Huntington's diseaseIs metabolic flexibility altered in multiple sclerosis patients?Altered Ca(2+) signaling in skeletal muscle fibers of the R6/2 mouse, a model of Huntington's diseaseTherapeutic approaches to preventing cell death in Huntington diseaseInvolvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtinThalamic metabolism and symptom onset in preclinical Huntington's disease.Low anaerobic threshold and increased skeletal muscle lactate production in subjects with Huntington's disease.HDAC inhibition imparts beneficial transgenerational effects in Huntington's disease mice via altered DNA and histone methylation.A ketogenic diet delays weight loss and does not impair working memory or motor function in the R6/2 1J mouse model of Huntington's diseaseDifferential effects of the Huntington's disease CAG mutation in striatum and cerebellum are quantitative not qualitative.Cardiac dysfunction in the R6/2 mouse model of Huntington's diseaseExperimental therapeutics in transgenic mouse models of Huntington's disease.The paradigm of Huntington's disease: therapeutic opportunities in neurodegeneration.Using advances in neuroimaging to detect, understand, and monitor disease progression in Huntington's disease.Fast-to-Slow Transition of Skeletal Muscle Contractile Function and Corresponding Changes in Myosin Heavy and Light Chain Formation in the R6/2 Mouse Model of Huntington's DiseaseA new Caenorhabditis elegans model of human huntingtin 513 aggregation and toxicity in body wall musclesA Metabolic Study of Huntington's Disease.Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction.Abnormal Weight and Body Mass Index in Children with Juvenile Huntington's Disease.Therapeutic perspectives for the treatment of Huntington's disease: treating the whole bodyEnhanced Ca(2+)-dependent glutamate release from astrocytes of the BACHD Huntington's disease mouse model.Impaired PGC-1alpha function in muscle in Huntington's disease.Mitochondrial approaches for neuroprotectionProgressive Cl- channel defects reveal disrupted skeletal muscle maturation in R6/2 Huntington's mice.The interrelationship between mitochondrial dysfunction and transcriptional dysregulation in Huntington disease.Brain networks in Huntington disease.
P2860
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P2860
Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy.
description
2000 nî lūn-bûn
@nan
2000年の論文
@ja
2000年学术文章
@wuu
2000年学术文章
@zh
2000年学术文章
@zh-cn
2000年学术文章
@zh-hans
2000年学术文章
@zh-my
2000年学术文章
@zh-sg
2000年學術文章
@yue
2000年學術文章
@zh-hant
name
Abnormal in vivo skeletal musc ...... torubropallidoluysian atrophy.
@en
Abnormal in vivo skeletal musc ...... torubropallidoluysian atrophy.
@nl
type
label
Abnormal in vivo skeletal musc ...... torubropallidoluysian atrophy.
@en
Abnormal in vivo skeletal musc ...... torubropallidoluysian atrophy.
@nl
prefLabel
Abnormal in vivo skeletal musc ...... torubropallidoluysian atrophy.
@en
Abnormal in vivo skeletal musc ...... torubropallidoluysian atrophy.
@nl
P2093
P1433
P1476
Abnormal in vivo skeletal musc ...... torubropallidoluysian atrophy.
@en
P2093
P356
10.1002/1531-8249(200007)48:1<72::AID-ANA11>3.3.CO;2-9
P577
2000-07-01T00:00:00Z