Mitochondrial quality control: a matter of life and death for neurons
TLDR
The role of the mitochondrial QC network for neuronal survival and neurodegeneration is discussed and mitochondrial proteases emerge as central regulators that coordinate different quality control pathways within an interconnected network of mechanisms.Abstract:
Neuronal survival critically depends on the integrity and functionality of mitochondria. A hierarchical system of cellular surveillance mechanisms protects mitochondria against stress, monitors mitochondrial damage and ensures the selective removal of dysfunctional mitochondrial proteins or organelles. Mitochondrial proteases emerge as central regulators that coordinate different quality control (QC) pathways within an interconnected network of mechanisms. A failure of this system causes neuronal loss in a steadily increasing number of neurodegenerative disorders, which include Parkinson's disease, spinocerebellar ataxia, spastic paraplegia and peripheral neuropathies. Here, we will discuss the role of the mitochondrial QC network for neuronal survival and neurodegeneration.read more
Citations
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Mitochondrial Reactive Oxygen Species (ROS) and ROS-Induced ROS Release
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Mitochondrial ROS Signaling in Organismal Homeostasis
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Mitochondrial rhomboid parl regulates cytochrome c release during apoptosis via OPA1 dependent cristae remodeling
Sara Cipolat,Tomasz Rudka,Dieter H. Hartmann,Costa,S. Serneels,Kathleen Craessaerts,Kristine Metzger,Luca Scorrano,Bart De Strooper +8 more
TL;DR: Parl-associated rhomboid-like (PARL-like) as mentioned in this paper is an inner mitochondrial membrane rhomboids of unknown function, whose yeast ortholog is involved in mitochondrial fusion.
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Parkin and PINK1 function in a vesicular trafficking pathway regulating mitochondrial quality control.
TL;DR: It is hypothesize that loss of this parkin‐ and PINK1‐dependent trafficking mechanism impairs the ability of mitochondria to selectively degrade oxidized and damaged proteins leading, over time, to the mitochondrial dysfunction noted in PD.
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