Autophagy in Polyglutamine Disease: Imposing Order on Disorder or Contributing to the Chaos?
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Summary
The role of autophagy in polyQ disorders and the therapeutic potential for autophagy modulation in these diseases are considered.
- Type
- review
- Published
- 2015-03-11
- Cited by
- 66
- References
- 86
- Access
- Open access
- OpenAlex
- https://openalex.org/W2072936799
- Semantic Scholar
- https://api.semanticscholar.org/CorpusID:20153280
Keywords
Autophagy, Biology, Cell biology, Proteostasis, TFEB
References
- Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease.
- Let-7 coordinately suppresses components of the amino acid sensing pathway to repress mTORC1 and induce autophagy
- Mutant protein in Huntington disease is resistant to proteolysis in affected brain
- Huntingtin Expression Stimulates Endosomal–Lysosomal Activity, Endosome Tubulation, and Autophagy
- Repeat expansion disease: Progress and puzzles in disease pathogenesis
- HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS
- Neurodegeneration by polyglutamine Atrophin is not rescued by induction of autophagy
- Deletion of the Huntingtin Polyglutamine Stretch Enhances Neuronal Autophagy and Longevity in Mice
- Proteome analysis of soluble nuclear proteins reveals that HMGB1/2 suppress genotoxic stress in polyglutamine diseases
- Development of Purkinje cell degeneration in a knockin mouse model reveals lysosomal involvement in the pathogenesis of SCA6
- Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation.
- Reinstating aberrant mTORC1 activity in Huntington's disease mice improves disease phenotypes
- CLEARance wars: PolyQ strikes back
- Genome-Wide Screen for Modifiers of Ataxin-3 Neurodegeneration in Drosophila
- Role of High Mobility Group Box 1 (HMGB1) in SCA17 Pathogenesis
- Post-translational modification of ataxin-7 at lysine-257 prevents autophagy-mediated turnover of an N-terminal caspase-7 cleavage fragment
- Disrupted Transforming Growth Factor-β Signaling in Spinal and Bulbar Muscular Atrophy
- IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome
- Proteasomal and autophagic degradative activities in spinal and bulbar muscular atrophy.
- The gene coding for PGC-1α modifies age at onset in Huntington's Disease
Cited by
- Mouse polyQ database: a new online resource for research using mouse models of neurodegenerative diseases
- Derailed Proteostasis as a Determinant of Cardiac Aging.
- Role of autophagy in chronic kidney diseases.
- Stabilization and Degradation Mechanisms of Cytoplasmic Ataxin-1
- Dopamine signaling promotes the xenobiotic stress response and protein homeostasis
- Implications of the cardiomyocyte stress response on protein homeostasis in atrial fibrillation
- Autophagy core machinery: overcoming spatial barriers in neurons
- A triazole derivative elicits autophagic clearance of polyglutamine aggregation in neuronal cells
- Discovery of Therapeutic Approaches for Polyglutamine Diseases: A Summary of Recent Efforts
- p62 links the autophagy pathway and the ubiqutin–proteasome system upon ubiquitinated protein degradation
- Motor Dysfunctions and Neuropathology in Mouse Models of Spinocerebellar Ataxia Type 2: A Comprehensive Review
- Nanoparticulate strategies for the treatment of polyglutamine diseases by halting the protein aggregation process†
- Noncoding RNAs in protein clearance pathways: implications in neurodegenerative diseases
- Progress toward the development of treatment of spinal and bulbar muscular atrophy
- RNA biology of disease-associated microsatellite repeat expansions
- ATXN1 intermediate-length polyglutamine expansions are associated with amyotrophic lateral sclerosis.
- The CAG-polyglutamine repeat diseases: a clinical, molecular, genetic, and pathophysiologic nosology.
- Pharmacological Therapies for Machado-Joseph Disease.
- X-Linked Spinal and Bulbar Muscular Atrophy: From Clinical Genetic Features and Molecular Pathology to Mechanisms Underlying Disease Toxicity.
- Molecular Targets and Therapeutic Strategies in Spinocerebellar Ataxia Type 7
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