Loss of LRPPRC causes ATP synthase deficiency

TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication

(2013) D. Milenkovic et al.   HUMAN MOLECULAR GENETICS

Human F1F0 ATP Synthase, Mitochondrial Ultrastructure and OXPHOS Impairment: A (Super-)Complex Matter?

(2013) Johann Habersetzer et al.   PLoS One

ATP synthase oligomerization: From the enzyme models to the mitochondrial morphology

(2012) Johan Habersetzer et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Variation in germline mtDNA heteroplasmy is determined prenatally but modified during subsequent transmission

(2012) Christoph Freyer et al.   NATURE GENETICS

Oligomycin frames a common drug-binding site in the ATP synthase

(2012) J. Symersky et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

The OASIS® HLB μElution plate as a one-step platform for manual high-throughput in-gel digestion of proteins and peptide desalting

(2012) Thomas Franz et al.   PROTEOMICS

LRPPRC mutation suppresses cytochrome oxidase activity by altering mitochondrial RNA transcript stability in a mouse model

(2011) Fenghao Xu et al.   BIOCHEMICAL JOURNAL

MTERF4 Regulates Translation by Targeting the Methyltransferase NSUN4 to the Mammalian Mitochondrial Ribosome

(2011) Yolanda Cámara et al.   Cell Metabolism

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

(2011) Benedetta Ruzzenente et al.   EMBO JOURNAL

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

Mice deleted for heart-type cytochrome c oxidase subunit 7a1 develop dilated cardiomyopathy

(2011) Maik Hüttemann et al.   MITOCHONDRION

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

The Bicoid Stability Factor Controls Polyadenylation and Expression of Specific Mitochondrial mRNAs in Drosophila melanogaster

(2011) Ana Bratic et al.   PLoS Genetics

Electron competition process in respiratory chain: Regulatory mechanisms and physiological functions

(2010) Michel Rigoulet et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

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

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

Mitochondrial ATP synthase deficiency due to a mutation in the ATP5E gene for the F1   subunit

(2010) J. A. Mayr et al.   HUMAN MOLECULAR GENETICS

Mitochondrial and Nuclear Genomic Responses to Loss ofLRPPRCExpression

(2010) Vishal M. Gohil et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Active proton leak in mitochondria: A new way to regulate substrate oxidation

(2009) Arnaud Mourier et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Methylation of 12S rRNA Is Necessary for In Vivo Stability of the Small Subunit of the Mammalian Mitochondrial Ribosome

(2009) Metodi D. Metodiev et al.   Cell Metabolism

Analysis of mouse models of cytochrome c oxidase deficiency owing to mutations in Sco2

(2009) Hua Yang et al.   HUMAN MOLECULAR GENETICS

The structure of the membrane extrinsic region of bovine ATP synthase

(2009) D. M. Rees et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Regulation of Mitochondrial Structure and Function by the F1Fo-ATPase Inhibitor Protein, IF1

(2008) Michelangelo Campanella et al.   Cell Metabolism

Dimer ribbons of ATP synthase shape the inner mitochondrial membrane

(2008) Mike Strauss et al.   EMBO JOURNAL

Yeast Cells Depleted in Atp14p Fail to Assemble Atp6p within the ATP Synthase and Exhibit Altered Mitochondrial Cristae Morphology

(2008) Vanessa Goyon et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Leigh and Leigh-Like Syndrome in Children and Adults

(2008) Josef Finsterer   PEDIATRIC NEUROLOGY