New Discoveries on Neurodegenerative Disorder
- Strong evidence has emerged to implicate disturbed mitochondrial fusion and fission as central pathological components underpinning a number of neurodegenerative disorders. Imbalances in mitochondrial fusion and fission affects respiratory chain function, mitochondrial quality control (mitophagy), and programmed cell death. The same cellular processes are also implicated in more-common complex neurodegenerative disorders, such as Alzheimer disease and Parkinson disease. This indicates a common pathological thread and a close relationship among mitochondrial structure, function and localization. (Nature Reviews Neurology, 2015)
- A mitochondrial fission arrest phenotype may cause elongated interconnected organelles, "mitochondria-on-a-string" (MOAS). Findings suggest that MOAS formation may occur at the final stages of fission process and was not associated with altered translocation of activated dynamin related protein 1 (Drp1) to mitochondria but with reduced GTPase activity. The findings on this research brings new attention to the major role of mitochondrial dynamics in regulating neuronal survival. (Scientific Reports, 2016)
- Mutations in Parkin and PINK1 cause an inherited early-onset form of Parkinson's disease. The two proteins function together in a mitochondrial quality control pathway. Here, a binding switch between phospho-ubiquitin (pUb) and the ubiquitin-like domain (Ubl) of Parkin was found to play a key role in the release of auto-inhibited ubiquitin ligase activity of Parkin. (The EMBO J, 2015)
Neurodegenerative Disorders
Mitochondria Implicated Neurodegenerative Disorder Pathways
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References |
Florence Burté, et.al. (2015). Nature Review Neurology. DOI: 10.1038/nrneurol.2014.228
Liang Zhang, et. al. (2016) Nature Scientific Reports, DOI: 10.1038/srep18725
Véronique Sauvé, et. al. (2015) The EMBO Journal. DOI: 10.15252/embj.201592237 |
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