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Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases
Hibiki Kawamata1, Giovanni Manfredi2
1Feil Family Brain and Mind Research Institute, Weill Cornell Medicine, New York, NY.
The Journal of Cell Biology
|November 24, 2017
Summary
Misfolded proteins in neurodegenerative diseases may harm mitochondria, impacting energy production. This review examines how protein aggregates affect mitochondrial function and oxidative phosphorylation.
Area of Science:
- Neuroscience
- Cell Biology
- Biochemistry
Background:
- Mitochondria are vital for nervous system functions, including metabolism and calcium regulation.
- Neurodegenerative diseases feature misfolded protein accumulation, potentially linked to mitochondrial dysfunction.
- Oxidative phosphorylation (OXPHOS) defects are observed in neurodegeneration, with misfolded proteins as a suspected cause.
Purpose of the Study:
- To critically review mechanisms linking misfolded proteins to mitochondria.
- To investigate the consequences of protein aggregation on mitochondrial oxidative phosphorylation (OXPHOS).
Main Methods:
- Literature review of studies on proteinopathies and mitochondrial function.
- Analysis of proposed molecular mechanisms of interaction.
Main Results:
- Misfolded proteins can associate with mitochondria, forming aggregates.
- These aggregates are implicated in mitochondrial dysfunction, particularly affecting OXPHOS.
Conclusions:
- The pathological role of proteinopathies within mitochondria requires further elucidation.
- Understanding these interactions is crucial for neurodegenerative disease research.
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