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Title: Mitochondrial rhomboid PARL regulates cytochrome c release during apoptosis via OPA1-dependent cristae remodeling
Authors: Cipolat, Sara *
Rudka, Tomasz *
Hartmann, Dieter *
Costa, Veronica
Serneels, Lutgarde
Craessaerts, Katleen
Metzger, Kristine
Frezza, Christian
Annaert, Wim
D'Adamio, Luciano
Derks, Carmen
Dejaegere, Tim
Pellegrini, Luca
D'Hooge, Rudi
Scorrano, Luca
De Strooper, Bart # ×
Issue Date: Jul-2006
Publisher: MIT Press
Series Title: Cell vol:126 issue:1 pages:163-175
Abstract: Rhomboids, evolutionarily conserved integral membrane proteases, participate in crucial signaling pathways. Presenilin-associated rhomboid-like (PARL) is an inner mitochondrial membrane rhomboid of unknown function, whose yeast ortholog is involved in mitochondrial fusion. Parl-/- mice display normal intrauterine development but from the fourth postnatal week undergo progressive multisystemic atrophy leading to cachectic death. Atrophy is sustained by increased apoptosis, both in and ex vivo. Parl-/- cells display normal mitochondrial morphology and function but are no longer protected against intrinsic apoptotic death stimuli by the dynamin-related mitochondrial protein OPA1. Parl-/- mitochondria display reduced levels of a soluble, intermembrane space (IMS) form of OPA1, and OPA1 specifically targeted to IMS complements Parl-/- cells, substantiating the importance of PARL in OPA1 processing. Parl-/- mitochondria undergo faster apoptotic cristae remodeling and cytochrome c release. These findings implicate regulated intramembrane proteolysis in controlling apoptosis.
URI: 
ISSN: 0092-8674
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Molecular Genetics Section (-)
Department of Human Genetics - miscellaneous
Laboratory for Biological Psychology
* (joint) first author
× corresponding author
# (joint) last author

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