Published in Cell on December 21, 2012
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Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy. Am J Hum Genet (2002) 1.73
A J-protein is an essential subunit of the presequence translocase-associated protein import motor of mitochondria. J Cell Biol (2003) 1.71
Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes. Am J Hum Genet (2011) 1.70
Mutations in C12orf65 in patients with encephalomyopathy and a mitochondrial translation defect. Am J Hum Genet (2010) 1.69
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The mitochondrial RNA-binding protein GRSF1 localizes to RNA granules and is required for posttranscriptional mitochondrial gene expression. Cell Metab (2013) 1.56
Taz1, an outer mitochondrial membrane protein, affects stability and assembly of inner membrane protein complexes: implications for Barth Syndrome. Mol Biol Cell (2005) 1.55
Tim50 maintains the permeability barrier of the mitochondrial inner membrane. Science (2006) 1.55
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Rcf1 mediates cytochrome oxidase assembly and respirasome formation, revealing heterogeneity of the enzyme complex. Cell Metab (2012) 1.48
Shy1 couples Cox1 translational regulation to cytochrome c oxidase assembly. EMBO J (2007) 1.47
Human SCO1 and SCO2 have independent, cooperative functions in copper delivery to cytochrome c oxidase. Hum Mol Genet (2004) 1.47
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Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1. Hum Mol Genet (2009) 1.45
Pex8p: an intraperoxisomal organizer of the peroxisomal import machinery. Mol Cell (2003) 1.45
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The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis. Cell Metab (2007) 1.38
Human Sco1 and Sco2 function as copper-binding proteins. J Biol Chem (2005) 1.37
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Pam17 is required for architecture and translocation activity of the mitochondrial protein import motor. Mol Cell Biol (2005) 1.34
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The presequence translocase-associated protein import motor of mitochondria. Pam16 functions in an antagonistic manner to Pam18. J Biol Chem (2004) 1.34
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The translocator maintenance protein Tam41 is required for mitochondrial cardiolipin biosynthesis. J Cell Biol (2008) 1.32
A role for Tim21 in membrane-potential-dependent preprotein sorting in mitochondria. Curr Biol (2006) 1.32
STED super-resolution microscopy reveals an array of MINOS clusters along human mitochondria. Proc Natl Acad Sci U S A (2013) 1.29
Reconstitution of mitochondria derived vesicle formation demonstrates selective enrichment of oxidized cargo. PLoS One (2012) 1.27
Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism. Nat Genet (2012) 1.26
Tmem16b is specifically expressed in the cilia of olfactory sensory neurons. Chem Senses (2010) 1.26
Prospect of preimplantation genetic diagnosis for heritable mitochondrial DNA diseases. Mol Hum Reprod (2003) 1.26
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Mitochondrial translocation contact sites: separation of dynamic and stabilizing elements in formation of a TOM-TIM-preprotein supercomplex. EMBO J (2003) 1.21
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Coa3 and Cox14 are essential for negative feedback regulation of COX1 translation in mitochondria. J Cell Biol (2010) 1.20
Mitochondrial import of the ADP/ATP carrier: the essential TIM complex of the intermembrane space is required for precursor release from the TOM complex. Mol Cell Biol (2002) 1.19
Mdm38 interacts with ribosomes and is a component of the mitochondrial protein export machinery. J Cell Biol (2006) 1.19
On the mechanism of preprotein import by the mitochondrial presequence translocase. Biochim Biophys Acta (2010) 1.19
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The A3243G tRNALeu(UUR) MELAS mutation causes amino acid misincorporation and a combined respiratory chain assembly defect partially suppressed by overexpression of EFTu and EFG2. Hum Mol Genet (2008) 1.17
A novel mutation in YARS2 causes myopathy with lactic acidosis and sideroblastic anemia. Hum Mutat (2012) 1.14
Mitochondria use different mechanisms for transport of multispanning membrane proteins through the intermembrane space. Mol Cell Biol (2003) 1.14
Dual function of Sdh3 in the respiratory chain and TIM22 protein translocase of the mitochondrial inner membrane. Mol Cell (2011) 1.14
Members of the E2D (UbcH5) family mediate the ubiquitination of the conserved cysteine of Pex5p, the peroxisomal import receptor. J Biol Chem (2008) 1.13
Radioactive labeling of mitochondrial translation products in cultured cells. Methods Mol Biol (2012) 1.11
Coupling of mitochondrial import and export translocases by receptor-mediated supercomplex formation. Cell (2013) 1.11
Tim22, the essential core of the mitochondrial protein insertion complex, forms a voltage-activated and signal-gated channel. Mol Cell (2002) 1.11
Distinct forms of mitochondrial TOM-TIM supercomplexes define signal-dependent states of preprotein sorting. Mol Cell Biol (2010) 1.11
Oxidative phosphorylation differences between mitochondrial DNA haplogroups modify the risk of Leber's hereditary optic neuropathy. Biochim Biophys Acta (2012) 1.11
Mutations in C12orf62, a factor that couples COX I synthesis with cytochrome c oxidase assembly, cause fatal neonatal lactic acidosis. Am J Hum Genet (2012) 1.08
Gimap3 regulates tissue-specific mitochondrial DNA segregation. PLoS Genet (2010) 1.08