The DOI matches its Crossref record; 139 of the 141 references it cites were found in a registry or an index.
Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders
Open accessAbstract
Lysosomal storage disorders (LSDs) are rare inherited debilitating and often fatal disorders. Caused by mutations affecting lysosomal proteins, LSDs are characterized by the accumulation of undegraded material in lysosomes and by lysosomal dysfunction. Although LSDs are multisystemic diseases, the majority display neurologic symptoms and neurodegeneration. Only recently has a role emerged for mitochondrial dysfunction in the pathophysiology of LSDs, suggesting an impact of lysosomal dysfunction on mitochondria. Moreover, mitochondrial damage may also cause lysosomal dysfunction, further supporting the activity of common signaling pathways and crosstalk between the two organelles. In this review we explore the mechanisms linking lysosomal and mitochondrial dysfunction to assess whether specific mitochondrial pathways represent a new therapeutic frontier in the management of LSDs.
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“In some circumstances, LMP appears to occur downstream of MOMP as a result of apoptotic signaling, de facto constituting an epiphenomenon of intrinsic apoptosis [757–759].”
From Molecular mechanisms of cell death: recommendations of the nomenclature committee on cell death 2018. (2018) · cites this paper as [759]
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1 retracted
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Metadata
- Authors
- Plotegher, N., & Duchen, M. R.
- Affiliations
- University College London (GB)
- Corresponding author
- Duchen, Michael R.
- Venue
- Trends in Molecular Medicine
- Publisher
- Elsevier BV
- Published
- 2017-02
- Volume
- 23
- Issue
- 2
- Pages
- 116-134
- ISSN
- 1471-4914 (ISSN-L)
- OpenAlex
- W2575523348
- Cited by
- 161
- References
- 141
- Citations by year
- 2026: 16 · 2025: 18 · 2024: 23 · 2023: 12 · 2022: 13 · 2021: 18 · 2020: 24 · 2019: 16 · 2018: 14 · 2017: 6
- FWCI
- 2.01
- Type
- journal article
- Language
- en
- Open access
- green
- hdl.handle.net
- Licence
- cc-by-nc-nd
- www.elsevier.com/tdm/userlicense/1.0/ (tdm)
- Locations
- Trends in Molecular Medicine (publishedVersion)
- PubMed (publishedVersion)
- Padua Research Archive (University of Padova) (submittedVersion, cc-by-nc-nd)
- UCL Discovery (University College London) (submittedVersion, cc-by-nc-nd)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Lysosomal Storage Disorders Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Glycogen Storage Diseases and Myoclonus
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Autophagy in Disease and Therapy
- Keywords
- lysosomal storage disease, mitochondrial dysfunction, neurodegeneration, lysosomal dysfunction, therapeutic target
- MeSH terms
- Animals, Humans, Lysosomes, Mitochondria, Mutation, Proteins, Lysosomal Storage Diseases, Neurodegenerative Diseases
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