Author : Gian-Luca McLelland
Publisher :
ISBN 13 :
Total Pages : pages
Book Rating : 4.:/5 (13 download)
Book Synopsis Parkinson's Disease Genes Orchestrate Mechanisms of Mitochondrial Quality Control by : Gian-Luca McLelland
Download or read book Parkinson's Disease Genes Orchestrate Mechanisms of Mitochondrial Quality Control written by Gian-Luca McLelland and published by . This book was released on 2018 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: "A functional mitochondrial network is cardinal to cellular fitness, and declines in mitochondrial function are linked to neurodegeneration, most notably the motor disorder Parkinson's disease. Two genes mutated in autosomal recessive juvenile parkinsonism, PARKIN and PINK1, work together to maintain mitochondrial health in vivo. Study over the past decade has revealed that PARKIN and PINK1 constitute a molecular system that recognizes and destroys damaged mitochondria via the autophagy pathway, in a process known as mitophagy. By removing dysfunctional organelles from the reticulum via this quality control mechanism, the PARKIN/PINK1 pathway protects the integrity of the mitochondrial network. The work comprised within this thesis delves into the mechanism of PINK1/PARKIN mitochondrial quality control. Firstly, it is shown that, in addition to mitophagy, PARKIN and PINK1 function in a second quality control mechanism that oversees the removal of damaged mitochondrial components, preserving mitochondrial function in the absence of organellar destruction. Selected, damaged mitochondrial cargo are extracted from the organelle via a class of mitochondrial-derived vesicles (MDVs), which are shuttled to the lysosome for turnover in a manner involving classical membrane fusion factors. Secondly, the work here demonstrates that uncoupling of mitochondria from the endoplasmic reticulum via the destruction of a tethering factor represents an important step in the mitophagic cascade. Together, these findings have furthered our understanding regarding the cellular toolkit that maintains mitochondrial health, and how its deterioration can lead to neurodegenerative disease." --