Loss of hepatic LRPPRC alters mitochondrial bioenergetics, regulation of permeability transition and trans-membrane ROS diffusion

Persistence of the mitochondrial permeability transition in the absence of subunit c of human ATP synthase

(2017) Jiuya He et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Inorganic polyphosphate (polyP) as an activator and structural component of the mitochondrial permeability transition pore

(2016) M. E. Solesio et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Normalization of NAD+Redox Balance as a Therapy for Heart FailureClinical Perspective

(2016) Chi Fung Lee et al.   CIRCULATION

Formation of mitochondrial-derived vesicles is an active and physiologically relevant mitochondrial quality control process in the cardiac system

(2016) Virgilio J. J. Cadete et al.   JOURNAL OF PHYSIOLOGY-LONDON

Specific requirements of nonbilayer phospholipids in mitochondrial respiratory chain function and formation

(2016) Charli D. Baker et al.   MOLECULAR BIOLOGY OF THE CELL

Increased Hepatic Fatty Acids Uptake and Oxidation by LRPPRC-Driven Oxidative Phosphorylation Reduces Blood Lipid Levels

(2016) Shi Lei et al.   Frontiers in Physiology

LRPPRCmutations cause early-onset multisystem mitochondrial disease outside of the French-Canadian population

(2015) Monika Oláhová et al.   BRAIN

Physiological and Pathological Roles of the Mitochondrial Permeability Transition Pore in the Heart

(2015) Jennifer Q. Kwong et al.   Cell Metabolism

From ATP to PTP and Back: A Dual Function for the Mitochondrial ATP Synthase

(2015) P. Bernardi et al.   CIRCULATION RESEARCH

Mechanical ventilation triggers abnormal mitochondrial dynamics and morphology in the diaphragm

(2015) Martin Picard et al.   JOURNAL OF APPLIED PHYSIOLOGY

Mitochondrial Vulnerability and Increased Susceptibility to Nutrient-Induced Cytotoxicity in Fibroblasts from Leigh Syndrome French Canadian Patients

(2015) Yan Burelle et al.   PLoS One

Tissue-specific responses to the LRPPRC founder mutation in French Canadian Leigh Syndrome

(2014) F. Sasarman et al.   HUMAN MOLECULAR GENETICS

Loss of LRPPRC causes ATP synthase deficiency

(2014) A. Mourier et al.   HUMAN MOLECULAR GENETICS

Channel Formation by Yeast F-ATP Synthase and the Role of Dimerization in the Mitochondrial Permeability Transition

(2014) Michela Carraro et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lipid landscapes and pipelines in membrane homeostasis

(2014) Joost C.M. Holthuis et al.   NATURE

An uncoupling channel within the c-subunit ring of the F1FO ATP synthase is the mitochondrial permeability transition pore

(2014) K. N. Alavian et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Dimers of mitochondrial ATP synthase form the permeability transition pore

(2013) V. Giorgio et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Lipids of mitochondria

(2013) Susanne E. Horvath et al.   PROGRESS IN LIPID RESEARCH

Inhibition of complex I regulates the mitochondrial permeability transition through a phosphate-sensitive inhibitory site masked by cyclophilin D

(2012) Bo Li et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Curvature, Lipid Packing, and Electrostatics of Membrane Organelles: Defining Cellular Territories in Determining Specificity

(2012) Joëlle Bigay et al.   DEVELOPMENTAL CELL

Phosphatidylethanolamine and Cardiolipin Differentially Affect the Stability of Mitochondrial Respiratory Chain Supercomplexes

(2012) Lena Böttinger et al.   JOURNAL OF MOLECULAR BIOLOGY

Peroxisome proliferator-activated receptor γ coactivator 1-α gene transfer restores mitochondrial biomass and improves mitochondrial calcium handling in post-necroticmdxmouse skeletal muscle

(2012) Richard Godin et al.   JOURNAL OF PHYSIOLOGY-LONDON

Structure of the yeast F1Fo-ATP synthase dimer and its role in shaping the mitochondrial cristae

(2012) K. M. Davies et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial aquaporin-8 knockdown in human hepatoma HepG2 cells causes ROS-induced mitochondrial depolarization and loss of viability

(2012) Maria Julia Marchissio et al.   TOXICOLOGY AND APPLIED PHARMACOLOGY

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

(2011) Benedetta Ruzzenente et al.   EMBO JOURNAL

LRP130 Protein Remodels Mitochondria and Stimulates Fatty Acid Oxidation

(2011) Lijun Liu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

LRPPRC mutations cause a phenotypically distinct form of Leigh syndrome with cytochrome c oxidase deficiency

(2011) F.-G. Debray et al.   JOURNAL OF MEDICAL GENETICS

Cyclophilin-D is dispensable for atrophy and mitochondrial apoptotic signalling in denervated muscle

(2011) Frederic N. Daussin et al.   JOURNAL OF PHYSIOLOGY-LONDON

Macromolecular organization of ATP synthase and complex I in whole mitochondria

(2011) K. M. Davies et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Assembly and oligomerization of human ATP synthase lacking mitochondrial subunits a and A6L

(2010) Ilka Wittig et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Comprehensive blood plasma lipidomics by liquid chromatography/quadrupole time-of-flight mass spectrometry

(2010) Koen Sandra et al.   JOURNAL OF CHROMATOGRAPHY A

LRPPRC and SLIRP Interact in a Ribonucleoprotein Complex That Regulates Posttranscriptional Gene Expression in Mitochondria

(2010) Florin Sasarman et al.   MOLECULAR BIOLOGY OF THE CELL

Disorders of mitochondrial function

(2009) François-Guillaume Debray et al.   CURRENT OPINION IN PEDIATRICS

Cyclophilin D Modulates Mitochondrial F0F1-ATP Synthase by Interacting with the Lateral Stalk of the Complex

(2009) Valentina Giorgio et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The antioxidant Trolox restores mitochondrial membrane potential and Ca2+-stimulated ATP production in human complex I deficiency

(2009) Felix Distelmaier et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Mitochondrial Disorders in the Nervous System

(2008) Salvatore DiMauro et al.   Annual Review of Neuroscience

Dimer ribbons of ATP synthase shape the inner mitochondrial membrane

(2008) Mike Strauss et al.   EMBO JOURNAL

Functional and stoichiometric analysis of subunit e in bovine heart mitochondrial F0F1ATP synthase

(2008) Elena Bisetto et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

The Mitochondrial Phosphate Carrier Interacts with Cyclophilin D and May Play a Key Role in the Permeability Transition

(2008) Anna W. C. Leung et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lipid extraction by methyl-tert-butyl ether for high-throughput lipidomics

(2008) Vitali Matyash et al.   JOURNAL OF LIPID RESEARCH

Increased expression and intramitochondrial translocation of cyclophilin-D associates with increased vulnerability of the permeability transition pore to stress-induced opening during compensated ventricular hypertrophy

(2008) Jimmy Matas et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

H2O2 induces rapid biophysical and permeability changes in the plasma membrane of Saccharomyces cerevisiae

(2007) Vanderlei Folmer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES