Copper supplementation restores cytochrome c oxidase assembly defect in a mitochondrial disease model of COA6 deficiency

Distilling Pathophysiology from Complex Disease Genetics

(2013) Aravinda Chakravarti et al.   CELL

Copper Import into the Mitochondrial Matrix in Saccharomyces cerevisiae Is Mediated by Pic2, a Mitochondrial Carrier Family Protein

(2013) Katherine E. Vest et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Opa1 Is Required for Proper Mitochondrial Metabolism in Early Development

(2013) Jennifer J. Rahn et al.   PLoS One

Mutations in C12orf62, a Factor that Couples COX I Synthesis with Cytochrome c Oxidase Assembly, Cause Fatal Neonatal Lactic Acidosis

(2012) Woranontee Weraarpachai et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Zebrafish in the Study of Early Cardiac Development

(2012) Jiandong Liu et al.   CIRCULATION RESEARCH

A mutation in the FAM36A gene, the human ortholog of COX20, impairs cytochrome c oxidase assembly and is associated with ataxia and muscle hypotonia

(2012) Radek Szklarczyk et al.   HUMAN MOLECULAR GENETICS

Intermembrane Space Proteome of Yeast Mitochondria

(2012) F.-Nora Vögtle et al.   MOLECULAR & CELLULAR PROTEOMICS

Mitochondrial disorders as windows into an ancient organelle

(2012) Scott B. Vafai et al.   NATURE

LRPPRC/SLIRP suppresses PNPase-mediated mRNA decay and promotes polyadenylation in human mitochondria

(2012) Takeshi Chujo et al.   NUCLEIC ACIDS RESEARCH

Iterative orthology prediction uncovers new mitochondrial proteins and identifies C12orf62 as the human ortholog of COX14, a protein involved in the assembly of cytochrome c oxidase

(2012) Radek Szklarczyk et al.   GENOME BIOLOGY

A Mutation in C2orf64 Causes Impaired Cytochrome c Oxidase Assembly and Mitochondrial Cardiomyopathy

(2011) Merei Huigsloot et al.   AMERICAN JOURNAL OF HUMAN GENETICS

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

(2011) Fenghao Xu et al.   BIOCHEMICAL JOURNAL

Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core

(2011) Ileana C. Soto et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

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

(2011) Benedetta Ruzzenente et al.   EMBO JOURNAL

Copper Metallochaperones

(2010) Nigel J. Robinson et al.   Annual Review of Biochemistry

Novel insights into the assembly and function of human nuclear-encoded cytochromecoxidase subunits 4, 5a, 6a, 7a and 7b

(2010) Daniela Fornuskova et al.   BIOCHEMICAL JOURNAL

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

Importing Mitochondrial Proteins: Machineries and Mechanisms

(2009) Agnieszka Chacinska et al.   CELL

Systematic Analysis of the Twin Cx9C Protein Family

(2009) Sebastian Longen et al.   JOURNAL OF MOLECULAR BIOLOGY

Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome

(2009) Woranontee Weraarpachai et al.   NATURE GENETICS

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology

(2008) David J. Pagliarini et al.   CELL

Mitochondrial copper metabolism and delivery to cytochromec oxidase

(2008) Darryl Horn et al.   IUBMB LIFE

Mapping the Functional Interaction of Sco1 and Cox2 in Cytochrome Oxidase Biogenesis

(2008) Kevin Rigby et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cmc1p Is a Conserved Mitochondrial Twin CX9C Protein Involved in Cytochrome c Oxidase Biogenesis

(2008) D. Horn et al.   MOLECULAR AND CELLULAR BIOLOGY