Cited by 163Lysosomal Storage Disorders ResearchGlycogen Storage Diseases and MyoclonusAutophagy in Disease and Therapy

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Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders

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Plotegher, Nicoletta, Duchen, Michael R.·2017·Trends in Molecular Medicine

DOI 10.1016/j.molmed.2016.12.003

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Abstract

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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Authors: Plotegher, N., & Duchen, M. R.
Affiliations: University College London (GB)
Corresponding author: Duchen, Michael R.
ORCID: Plotegher, Nicoletta
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)
DOI: 10.1016/j.molmed.2016.12.003
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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Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders

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