Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders.

doi:10.1016/j.molmed.2016.12.003

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Article

Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders.

Trends in molecular medicine - 1 Feb 2017

Plotegher Nicoletta, Duchen Michael R

Abstract excerpt

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...

Topics

Animals
Humans
Lysosomal Storage Diseases
Lysosomes
Mitochondria
Mutation
Neurodegenerative Diseases
Proteins

Source

Open in PubMed

Identifiers

PMID: 28111024
DOI: 10.1016/j.molmed.2016.12.003

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