Molecular Biology

Protecting Mitochondria

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Science  07 Feb 2014:
Vol. 343, Issue 6171, pp. 581
DOI: 10.1126/science.343.6171.581-a
CREDIT: G. MCLELLAND ET AL., THE EMBO JOURNAL (20 JANUARY 2014) © 2014 WILEY-VCH VERLAG GMBH & CO. KGAA. REPRODUCED WITH PERMISSION.

The power plant of the cell, the mitochondrion, can be a hostile environment in which proteins or the whole organelle can become damaged. To prevent further disruption of the cell, whole damaged mitochondria can be engulfed and degraded by autophagosomes, and the failure of this process may cause neurodegeneration. The Parkin and PINK1 genes—mutations of which are linked to neurodegenerative Parkinson's disease—function in this process. McLelland et al. describe a less dramatic protection mechanism in which proteins damaged by overproduction of reactive oxygen species in the mitochondria are carted in membrane vesicles to lysosomes. Like the wholesale remodeling of mitochondria themselves, this vesicle-mediated quality-control mechanism required the PINK1 and Parkin proteins. The authors propose that the protective actions of Parkin and PINK1 may play out in two stages: a more rapid one in which damaged proteins can be selectively removed in vesicles, and, in cases where damage is advanced, the recycling of the full organelles by mitophagy.

EMBO J. 10.1002/embj.201385902 (2014).

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