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Mechanisms of protein toxicity in neurodegenerative diseases

Title
Mechanisms of protein toxicity in neurodegenerative diseases
Authors
Chung, Chang GeonLee, HyosangLee, Sung Bae
DGIST Authors
Chung, Chang Geon; Lee, HyosangLee, Sung Bae
Issue Date
2018-09
Citation
Cellular and Molecular Life Sciences, 75(17), 3159-3180
Type
Article
Article Type
Review
Author Keywords
Parkinson&aposs diseaseAlzheimer&aposs diseaseHuntington&aposs diseasePolyglutamine diseasesLou Gehrig&aposs diseaseAmyotrophic lateral sclerosisFrontotemporal dementiaStress granulesProtein inclusions
Keywords
SELECTIVE NEURONAL VULNERABILITYPATHOLOGICAL ALPHA-SYNUCLEINCHAPERONE-MEDIATED AUTOPHAGYUBIQUITIN-PROTEASOME SYSTEMAMYLOID PRECURSOR PROTEINAXONAL-TRANSPORT DEFECTSSTRESS GRANULE DYNAMICSCREB-BINDING PROTEINAMYOTROPHIC-LATERAL-SCLEROSISSPORADIC PARKINSONS-DISEASE
ISSN
1420-682X
Abstract
Protein toxicity can be defined as all the pathological changes that ensue from accumulation, mis-localization, and/or multimerization of disease-specific proteins. Most neurodegenerative diseases manifest protein toxicity as one of their key pathogenic mechanisms, the details of which remain unclear. By systematically deconstructing the nature of toxic proteins, we aim to elucidate and illuminate some of the key mechanisms of protein toxicity from which therapeutic insights may be drawn. In this review, we focus specifically on protein toxicity from the point of view of various cellular compartments such as the nucleus and the mitochondria. We also discuss the cell-to-cell propagation of toxic disease proteins that complicates the mechanistic understanding of the disease progression as well as the spatiotemporal point at which to therapeutically intervene. Finally, we discuss selective neuronal vulnerability, which still remains largely enigmatic. © 2018, The Author(s).
URI
http://hdl.handle.net/20.500.11750/9223
DOI
10.1007/s00018-018-2854-4
Publisher
Birkhauser Verlag AG
Related Researcher
  • Author Lee, Sung Bae Laboratory of Neurodegenerative Diseases and Aging
  • Research Interests Cellular mechanism of neurodegenerative diseases; Neuronal maintenance and remodeling; 퇴행성 뇌질환의 세포기전; 신경계 유지 및 리모델링 연구
Files:
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Collection:
Department of Brain and Cognitive SciencesLaboratory of Affective Neuroscience1. Journal Articles
Department of Brain and Cognitive SciencesLaboratory of Neurodegenerative Diseases and Aging1. Journal Articles


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