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Title: Mis-targeting of the mitochondrial protein LIPT2 leads to apoptotic cell death
Authors: Bernardinelli, Emanuele
Costa, Roberta
Scantamburlo, Giada
To, Janet
Morabito, Rossana
Nofziger, Charity
Doerrier, Carolina
Krumschnabel, Gerhard
Paulmichl, Markus
Dossena, Silvia
Keywords: Mitochondrial Protein
Issue Date: 2017
Source: Bernardinelli, E., Costa, R., Scantamburlo, G., To, J., Morabito, R., Nofziger, C., et al. (2017). Mis-targeting of the mitochondrial protein LIPT2 leads to apoptotic cell death. PLOS ONE, 12(6), e0179591-.
Series/Report no.: PLOS ONE
Abstract: Lipoyl(Octanoyl) Transferase 2 (LIPT2) is a protein involved in the post-translational modification of key energy metabolism enzymes in humans. Defects of lipoic acid synthesis and transfer start to emerge as causes of fatal or severe early-onset disease. We show that the first 31 amino acids of the N-terminus of LIPT2 represent a mitochondrial targeting sequence and inhibition of the transit of LIPT2 to the mitochondrion results in apoptotic cell death associated with activation of the apoptotic volume decrease (AVD) current in normotonic conditions, as well as over-activation of the swelling-activated chloride current (IClswell), mitochondrial membrane potential collapse, caspase-3 cleavage and nuclear DNA fragmentation. The findings presented here may help elucidate the molecular mechanisms underlying derangements of lipoic acid biosynthesis.
DOI: 10.1371/journal.pone.0179591
Rights: © 2017 Bernardinelli et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Fulltext Permission: open
Fulltext Availability: With Fulltext
Appears in Collections:SBS Journal Articles

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