Nonsense mutations in the COX1 subunit impair the stability of respiratory chain complexes rather than their assembly

Assembly factors and ATP-dependent proteases in cytochrome c oxidase biogenesis

(2010) Lukas Stiburek et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The Carboxyl-terminal End of Cox1 Is Required for Feedback Assembly Regulation of Cox1 Synthesis inSaccharomyces cerevisiaeMitochondria

(2010) Miguel Shingú-Vázquez et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28

(2010) Daniela Di Bella et al.   NATURE GENETICS

A Novel Mutation in the SCO2 Gene in a Neonate With Early-Onset Cardioencephalomyopathy

(2010) Kairit Joost et al.   PEDIATRIC NEUROLOGY

Isolation of functional pure mitochondria by superparamagnetic microbeads

(2009) Hue-Tran Hornig-Do et al.   ANALYTICAL BIOCHEMISTRY

Role of nuclear-encoded subunit Vb in the assembly and stability of cytochromecoxidase complex: implications in mitochondrial dysfunction and ROS production

(2009) Domenico Galati et al.   BIOCHEMICAL JOURNAL

Assembly of nuclear DNA-encoded subunits into mitochondrial complex IV, and their preferential integration into supercomplex forms in patient mitochondria

(2009) Michael Lazarou et al.   FEBS Journal

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

(2009) Hua Yang et al.   HUMAN MOLECULAR GENETICS

Regulation of OPA1 processing and mitochondrial fusion bym-AAA protease isoenzymes and OMA1

(2009) Sarah Ehses et al.   JOURNAL OF CELL BIOLOGY

Dual Functions of Mss51 Couple Synthesis of Cox1 to Assembly of Cytochrome c Oxidase in Saccharomyces cerevisiae Mitochondria

(2009) Xochitl Perez-Martinez et al.   MOLECULAR BIOLOGY OF THE CELL

Severe Infantile Encephalomyopathy Caused by a Mutation in COX6B1, a Nucleus-Encoded Subunit of Cytochrome C Oxidase

(2008) Valeria Massa et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Identification of novel mutations in five patients with mitochondrial encephalomyopathy

(2008) Lucia Valente et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Assembly of the oxidative phosphorylation system in humans: What we have learned by studying its defects

(2008) Erika Fernández-Vizarra et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Quality control of mitochondria: protection against neurodegeneration and ageing

(2008) Takashi Tatsuta et al.   EMBO JOURNAL

Complex I Function Is Defective in Complex IV-deficientCaenorhabditis elegans

(2008) Wichit Suthammarak et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hereditary spastic paraplegia: clinical features and pathogenetic mechanisms

(2008) Sara Salinas et al.   LANCET NEUROLOGY

Electrophoresis techniques to investigate defects in oxidative phosphorylation

(2008) Maria Antonietta Calvaruso et al.   METHODS

Respiratory Active Mitochondrial Supercomplexes

(2008) Rebeca Acín-Pérez et al.   MOLECULAR CELL

Neutral red uptake assay for the estimation of cell viability/cytotoxicity

(2008) Guillermo Repetto et al.   Nature Protocols

Forced extraction of targeted components from complex macromolecular assemblies

(2008) S. D. Moore et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA