The DOI matches its Crossref record; 76 of the 76 references it cites were found in a registry or an index.
The roles of intracellular protein-degradation pathways in neurodegeneration
Abstract
Many late-onset neurodegenerative diseases, including Parkinson's disease and Huntington's disease, are associated with the formation of intracellular aggregates by toxic proteins. It is therefore crucial to understand the factors that regulate the steady-state levels of these 'toxins', at both the synthetic and degradation stages. The degradation pathways acting on such aggregate-prone cytosolic proteins include the ubiquitin-proteasome system and macroautophagy. Dysfunction of the ubiquitin-proteasome or macroautophagy pathways might contribute to the pathology of various neurodegenerative conditions. However, enhancing macroautophagy with drugs such as rapamycin could offer a tractable therapeutic strategy for a number of these diseases.
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In the citing paper
“Currently, accumulating evidence suggests that autophagy is deregulated in neurodegenerative diseases and may play key roles in the etiology of these pathologies (Rubinsztein, 2006).”
From Downregulation of MicroRNA-193b-3p Promotes Autophagy and Cell Survival by Targeting TSC1/mTOR Signaling in NSC-34 Cells (2017) · cites this paper as Rubinsztein, 2006
In the citing paper
“Currently, accumulating evidence suggests that autophagy is deregulated in neurodegenerative diseases and may play key roles in the etiology of these pathologies (Rubinsztein, 2006).”
From Downregulation of MicroRNA-193b-3p Promotes Autophagy and Cell Survival by Targeting TSC1/mTOR Signaling in NSC-34 Cells (2017) · cites this paper as Rubinsztein, 2006
In the citing paper
“neurons degrade these aggregates only through the ubiquitin-proteasome system, or the autophagy-lysosomal pathway6.”
From Retromer stabilization results in neuroprotection in a model of Amyotrophic Lateral Sclerosis (2020) · cites this paper as [6]
In the citing paper
“Aging-associated expression changes in genes associated with mitochondrial function, protein metabolism, and immune response have been identified in several tissues (173) and are consistent with some proposed mechanisms for late-onset neurodegenerative diseases (98, 113, 124, 166).”
From Unraveling a Multifactorial Late-Onset Disease: From Genetic Susceptibility to Disease Mechanisms for Age-Related Macular Degeneration (2009) · cites this paper as [124]
In the citing paper
“aging-associated decline in these pathways can lead to protein aggregates that can compromise biochemical functions and cellular health (113, 124).”
From Unraveling a Multifactorial Late-Onset Disease: From Genetic Susceptibility to Disease Mechanisms for Age-Related Macular Degeneration (2009) · cites this paper as [124]
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Metadata
- Authors
- Rubinsztein, D. C.
- Affiliations
- University of Cambridge (GB)
- Corresponding author
- Rubinsztein, David C.
- Venue
- Nature
- Published
- 2006-10
- Volume
- 443
- Issue
- 7113
- Pages
- 780-786
- ISSN
- 0028-0836 (ISSN-L)
- 1476-4687
- OpenAlex
- W2025714022
- Cited by
- 1,625
- References
- 76
- Citations by year
- 2026: 21 · 2025: 40 · 2024: 35 · 2023: 44 · 2022: 68 · 2021: 66 · 2020: 74 · 2019: 79 · 2018: 64 · 2017: 89 · 2016: 91 · 2015: 114 · 2014: 106 · 2013: 116 · 2012: 119
- FWCI
- 12.03
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature (publishedVersion)
- PubMed (publishedVersion)
- RePEc: Research Papers in Economics (submittedVersion)
- Funders
- Wellcome Trust
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Autophagy in Disease and Therapy
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ubiquitin and proteasome pathways
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Keywords
- neurodegeneration, protein degradation, ubiquitin-proteasome system, macroautophagy, protein aggregates, Parkinson's disease, Huntington's disease, rapamycin, treatment options
- MeSH terms
- Huntingtin Protein, Animals, Autophagy, Humans, Lysosomes, Nerve Tissue Proteins, Nuclear Proteins, Neurodegenerative Diseases, Ubiquitin, Proteasome Endopeptidase Complex
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