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Semin Cell Dev Biol. 2016 Dec;60:19-28. doi: 10.1016/j.semcdb.2016.07.034. Epub 2016 Aug 5.

PARL: The mitochondrial rhomboid protease.

Author information

1
VIB Center for the Biology of Disease, O&N4 Herestraat 49 box 602, 3000, Leuven, Belgium; KU Leuven Center for Human Genetics, O&N4 Herestraat 49 box 602, 3000, Leuven, Belgium.
2
VIB Center for the Biology of Disease, O&N4 Herestraat 49 box 602, 3000, Leuven, Belgium; KU Leuven Center for Human Genetics, O&N4 Herestraat 49 box 602, 3000, Leuven, Belgium; UCL Institute of Neurology, University College London, WC1N 3BG, UK. Electronic address: bart.destrooper@cme.vib-kuleuven.be.

Abstract

The rhomboid family comprises evolutionary conserved intramembrane proteases involved in a wide spectrum of biologically relevant activities. A mitochondrion-localized rhomboid, called PARL in mammals, and conserved in yeast and Drosophila as RBD1/PCP1 and rho-7, respectively, plays an indispensable role in cell homeostasis as illustrated by the severe phenotypes caused by its genetic ablation in the various investigated species. Although several substrates of PARL have been proposed to explain these phenotypes, there remains a lot of controversy in this important area of research. We review here the putative functions and substrates of PARL and its orthologues in different species, highlighting areas of uncertainty, and discuss its potential involvement in some prevalent diseases such as type II diabetes and Parkinson's disease.

KEYWORDS:

Cell death; Metabolism; Mitochondria; PARL; Protease; Rhomboid

PMID:
27502471
DOI:
10.1016/j.semcdb.2016.07.034
[Indexed for MEDLINE]

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