(Q34600372)

3 August 2017

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title

Defects in cytochrome oxidase assembly in humans: lessons from yeast (English)

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3 August 2017

Jennifer M Zee

1

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3 August 2017

D Moira Glerum

series ordinal

2

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3 August 2017

publication date

1 December 2006

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Biochemistry and Cell Biology

3 August 2017

published in

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3 August 2017

volume

84

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3 August 2017

issue

6

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3 August 2017

page(s)

859-869

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Yeast cox17 solution structure and Copper(I) binding

3 August 2017

cites work

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Molecular analysis of cytochrome c oxidase deficiency in Leigh's syndrome

21 January 2018

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Isolation of a cDNA encoding the human homolog of COX17, a yeast gene essential for mitochondrial copper recruitment

21 January 2018

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Mutations in COX10 result in a defect in mitochondrial heme A biosynthesis and account for multiple, early-onset clinical phenotypes associated with isolated COX deficiency

21 January 2018

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Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy

21 January 2018

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Folding studies of Cox17 reveal an important interplay of cysteine oxidation and copper binding

21 January 2018

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Solution structure of Cox11, a novel type of beta-immunoglobulin-like fold involved in CuB site formation of cytochrome c oxidase

21 January 2018

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Mutational analysis of the Saccharomyces cerevisiae cytochrome c oxidase assembly protein Cox11p

21 January 2018

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Mss51p and Cox14p jointly regulate mitochondrial Cox1p expression in Saccharomyces cerevisiae

21 January 2018

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Involvement of mitochondrial ferredoxin and Cox15p in hydroxylation of heme O.

21 January 2018

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Mitochondrial ferredoxin is required for heme A synthesis in Saccharomyces cerevisiae

21 January 2018

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COX23, a homologue of COX17, is required for cytochrome oxidase assembly

21 January 2018

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Purification, characterization, and localization of yeast Cox17p, a mitochondrial copper shuttle

21 January 2018

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Purification and characterization of yeast Sco1p, a mitochondrial copper protein

21 January 2018

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Cloning of a human gene involved in cytochrome oxidase assembly by functional complementation of an oxa1- mutation in Saccharomyces cerevisiae

21 January 2018

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Interactions among three proteins that specifically activate translation of the mitochondrial COX3 mRNA in Saccharomyces cerevisiae

21 January 2018

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Identification of novel hemes generated by heme A synthase: evidence for two successive monooxygenase reactions

21 January 2018

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Heme A synthase does not incorporate molecular oxygen into the formyl group of heme A.

21 January 2018

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COX16 encodes a novel protein required for the assembly of cytochrome oxidase in Saccharomyces cerevisiae

21 January 2018

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Functional analysis of the domains in Cox11.

21 January 2018

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Assembly of cytochrome c oxidase within the mitochondrion

21 January 2018

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A role for Pet100p in the assembly of yeast cytochrome c oxidase: interaction with a subassembly that accumulates in a pet100 mutant.

21 January 2018

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Copper trafficking to the mitochondrion and assembly of copper metalloenzymes

21 January 2018

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Genetic heterogeneity in Leigh syndrome.

21 January 2018

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An investigation of the metabolism of isoleucine to active Amyl alcohol in Saccharomyces cerevisiae

21 January 2018

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Copper chaperones: personal escorts for metal ions

21 January 2018

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Human recombinant mutated forms of the mitochondrial COX assembly Sco2 protein differ from wild-type in physical state and copper binding capacity

21 January 2018

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Reversion of hypertrophic cardiomyopathy in a patient with deficiency of the mitochondrial copper binding protein Sco2: is there a potential effect of copper?

21 January 2018

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Isolation of a human cDNA for heme A:farnesyltransferase by functional complementation of a yeast cox10 mutant

21 January 2018

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Affinity purification of yeast cytochrome oxidase with biotinylated subunits 4, 5, or 6.

21 January 2018

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Characterization of cytochrome-c oxidase mutants in human fibroblasts

21 January 2018

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Characterization of COX17, a yeast gene involved in copper metabolism and assembly of cytochrome oxidase

21 January 2018

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Oxa1p, an essential component of the N-tail protein export machinery in mitochondria

21 January 2018

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Identification of Cox20p, a novel protein involved in the maturation and assembly of cytochrome oxidase subunit 2.

21 January 2018

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Biogenesis of cytochrome oxidase-sophisticated assembly lines in the mitochondrial inner membrane

21 January 2018

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Cox11p is required for stable formation of the Cu(B) and magnesium centers of cytochrome c oxidase

21 January 2018

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Analysis of COX2 mutants reveals cytochrome oxidase subassemblies in yeast

21 January 2018

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Specific copper transfer from the Cox17 metallochaperone to both Sco1 and Cox11 in the assembly of yeast cytochrome C oxidase.

21 January 2018

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Human Sco1 and Sco2 function as copper-binding proteins

21 January 2018

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Mutations in SCO2 are associated with a distinct form of hypertrophic cardiomyopathy and cytochrome c oxidase deficiency

21 January 2018

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Homozygosity (E140K) in SCO2 causes delayed infantile onset of cardiomyopathy and neuropathy

21 January 2018

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Cytochrome c oxidase deficiency due to mutations in SCO2, encoding a mitochondrial copper-binding protein, is rescued by copper in human myoblasts

21 January 2018

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A microdeletion in cytochrome c oxidase (COX) subunit III associated with COX deficiency and recurrent myoglobinuria

21 January 2018

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Evidence for the association of yeast mitochondrial ribosomes with Cox11p, a protein required for the Cu(B) site formation of cytochrome c oxidase

21 January 2018

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Biogenesis of cytochrome c oxidase

21 January 2018

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New developments in mitochondrial assembly

21 January 2018

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Human SCO1 and SCO2 have independent, cooperative functions in copper delivery to cytochrome c oxidase

21 January 2018

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Biochemical and molecular analysis of cytochrome c oxidase deficiency in Leigh's syndrome

21 January 2018

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SHY1, the yeast homolog of the mammalian SURF-1 gene, encodes a mitochondrial protein required for respiration

21 January 2018

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Cox17 Is Functional When Tethered to the Mitochondrial Inner Membrane

21 January 2018

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PrrC from Rhodobacter sphaeroides, a homologue of eukaryotic Sco proteins, is a copper-binding protein and may have a thiol-disulfide oxidoreductase activity.

21 January 2018

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Sequence and chromosomal localization of two PET genes required for cytochrome c oxidase assembly in Saccharomyces cerevisiae

21 January 2018

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A disulfide relay system in the intermembrane space of mitochondria that mediates protein import

21 January 2018

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Identification of a gene causing human cytochrome c oxidase deficiency by integrative genomics

21 January 2018

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A single cell complementation class is common to several cases of cytochrome c oxidase-defective Leigh's syndrome.

21 January 2018

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Assembly of cytochrome-c oxidase in cultured human cells

21 January 2018

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Characterization of COX19, a widely distributed gene required for expression of mitochondrial cytochrome oxidase

21 January 2018

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Mba1, a membrane-associated ribosome receptor in mitochondria

21 January 2018

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Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene

21 January 2018

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Human members of the SCO1 gene family: complementation analysis in yeast and intracellular localization

21 January 2018

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Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain

21 January 2018

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Mutagenesis reveals a specific role for Cox17p in copper transport to cytochrome oxidase.

21 January 2018

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Characterization and localization of human COX17, a gene involved in mitochondrial copper transport

21 January 2018

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The role of protein degradation in mitochondrial function and biogenesis.

21 January 2018

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Human cytochrome oxidase deficiency

21 January 2018

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Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease

21 January 2018

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Multiple interactions involving the amino-terminal domain of yeast mtRNA polymerase determine the efficiency of mitochondrial protein synthesis

21 January 2018

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Novel SURF1 mutation in a child with subacute encephalopathy and without the radiological features of Leigh Syndrome

21 January 2018

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Cox18p is required for export of the mitochondrially encoded Saccharomyces cerevisiae Cox2p C-tail and interacts with Pnt1p and Mss2p in the inner membrane.

21 January 2018

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A direct repeat is a hotspot for large-scale deletion of human mitochondrial DNA.

21 January 2018

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SCO1, a yeast nuclear gene essential for accumulation of mitochondrial cytochrome c oxidase subunit II.

21 January 2018

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Cytochrome c oxidase deficiency

21 January 2018

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Bacillus subtilis CtaA and CtaB function in haem A biosynthesis.

21 January 2018

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Novel SCO2 mutation (G1521A) presenting as a spinal muscular atrophy type I phenotype

21 January 2018

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Mutations of SURF-1 in Leigh disease associated with cytochrome c oxidase deficiency

21 January 2018

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The whole structure of the 13-subunit oxidized cytochrome c oxidase at 2.8 A

21 January 2018

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A mutation in the human heme A:farnesyltransferase gene (COX10 ) causes cytochrome c oxidase deficiency

21 January 2018

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Mutations in SURF1 are not specifically associated with Leigh syndrome

21 January 2018

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Crystal structure of human SCO1: implications for redox signaling by a mitochondrial cytochrome c oxidase "assembly" protein

21 January 2018

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The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA

21 January 2018

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Expression and functional analysis of SURF1 in Leigh syndrome patients with cytochrome c oxidase deficiency

21 January 2018

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Identification of a disulfide switch in BsSco, a member of the Sco family of cytochrome c oxidase assembly proteins

21 January 2018

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X-ray structure and reaction mechanism of bovine heart cytochrome c oxidase

21 January 2018

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SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome

21 January 2018

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Copper supplementation restores cytochrome c oxidase activity in cultured cells from patients with SCO2 mutations

21 January 2018

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Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies

21 January 2018

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21 January 2018

Identifiers

DOI

10.1139/O06-201

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3 August 2017

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