Broad activation of the Parkin pathway induces synaptic mitochondrial deficits in early tauopathy
Yu Young Jeong1, Sinsuk Han1, Nuo Jia1
1Division of Life Science, Department of Cell Biology and Neuroscience, School of Arts and Sciences, Rutgers, The State University of New Jersey, Piscataway, NJ 08854, USA.
Abstract:
Mitochondrial defects are a hallmark of early pathophysiology in Alzheimer's disease, with pathologically phosphorylated tau reported to induce mitochondrial toxicity. Mitophagy constitutes a key pathway in mitochondrial quality control by which damaged mitochondria are targeted for autophagy. However, few details are known regarding the intersection of mitophagy and pathologies in tauopathy. Here, by applying biochemical and cell biological approaches including time-lapse confocal imaging in live tauopathy neurons, combined with gene rescue experiments via stereotactic injections of adeno-associated virus particles into tauopathy mouse brains, electrophysiological recordings and behavioural tests, we demonstrate for the first time that mitochondrial distribution deficits at presynaptic terminals are an early pathological feature in tauopathy brains. Furthermore, Parkin-mediated mitophagy is extensively activated in tauopathy neurons, which accelerates mitochondrial Rho GTPase 1 (Miro1) turnover and consequently halts Miro1-mediated mitochondrial anterograde movement towards synaptic terminals. As a result, mitochondrial supply at tauopathy synapses is disrupted, impairing synaptic function. Strikingly, increasing Miro1 levels restores the synaptic mitochondrial population by enhancing mitochondrial anterograde movement and thus reverses tauopathy-associated synaptic failure. In tauopathy mouse brains, overexpression of Miro1 markedly elevates synaptic distribution of mitochondria and protects against synaptic damage and neurodegeneration, thereby counteracting impairments in learning and memory as well as synaptic plasticity. Taken together, our study reveals that activation of the Parkin pathway triggers an unexpected effect-depletion of mitochondria from synaptic terminals, a characteristic feature of early tauopathy. We further provide new mechanistic insights into how parkin activation-enhanced Miro1 degradation and impaired mitochondrial anterograde transport drive tauopathy-linked synaptic pathogenesis and establish a foundation for future investigations into new therapeutic strategies to prevent synaptic deterioration in Alzheimer's disease and other tauopathies.
Insights
Mitochondrial defects in Alzheimer's disease are linked to tau pathology. Activated mitophagy depletes synaptic mitochondria by degrading Miro1, impairing function, but restoring Miro1 levels reverses this damage.
Area of Science:
- Neuroscience
- Cell Biology
- Mitochondrial Biology
Background:
- Mitochondrial dysfunction is an early indicator in Alzheimer's disease (AD) pathophysiology.
- Pathologically phosphorylated tau contributes to mitochondrial toxicity.
- Mitophagy is crucial for mitochondrial quality control, but its role in tauopathies is unclear.
Purpose of the Study:
- To investigate the intersection of mitophagy and tauopathy pathologies.
- To identify early pathological features of mitochondrial distribution in tauopathy.
- To elucidate the mechanisms underlying synaptic mitochondrial deficits in tauopathy.
Main Methods:
- Biochemical and cell biological approaches.
- Time-lapse confocal imaging in live tauopathy neurons.
- Gene rescue via adeno-associated virus (AAV) delivery in mouse models.
- Electrophysiological recordings and behavioral tests.
Main Results:
- Mitochondrial distribution deficits at presynaptic terminals are an early feature of tauopathy.
- Parkin-mediated mitophagy is activated, accelerating Miro1 turnover and halting mitochondrial anterograde transport.
- Increased Miro1 levels restore synaptic mitochondria, enhance anterograde movement, and reverse synaptic failure.
- Miro1 overexpression in tauopathy mouse brains protects against synaptic damage, neurodegeneration, and cognitive deficits.
Conclusions:
- Activated Parkin pathway unexpectedly leads to synaptic mitochondrial depletion in early tauopathy.
- Parkin-enhanced Miro1 degradation and impaired mitochondrial transport drive tauopathy-linked synaptic pathogenesis.
- Restoring Miro1 levels represents a potential therapeutic strategy for synaptic protection in Alzheimer's disease and tauopathies.
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