Gene Symbol: Cox10
Description: cytochrome c oxidase assembly protein 10
Alias: 2410004F01Rik, AU042636, protoheme IX farnesyltransferase, mitochondrial, COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase, heme O synthase
Species: mouse
Products:     Cox10

Top Publications

  1. Fukui H, Diaz F, Garcia S, Moraes C. Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease. Proc Natl Acad Sci U S A. 2007;104:14163-8 pubmed
    ..A neuron-specific COX-deficient mouse was generated by the Cre-loxP system, in which the COX10 gene was deleted by a CamKIIalpha promoter-driven Cre-recombinase...
  2. Peralta S, Garcia S, Yin H, Arguello T, Diaz F, Moraes C. Sustained AMPK activation improves muscle function in a mitochondrial myopathy mouse model by promoting muscle fiber regeneration. Hum Mol Genet. 2016;25:3178-3191 pubmed publisher
    ..whether prolonged AICAR treatment is beneficial in a mouse model of slowly progressing mitochondrial myopathy (Cox10-Mef2c-Cre), and whether the compensatory mechanism is indeed an increase in mitochondrial biogenesis...
  3. Tyynismaa H, Carroll C, Raimundo N, Ahola Erkkilä S, Wenz T, Ruhanen H, et al. Mitochondrial myopathy induces a starvation-like response. Hum Mol Genet. 2010;19:3948-58 pubmed publisher
    ..These results may have important implications for understanding the metabolic consequences of mitochondrial myopathies. ..
  4. Diaz F, Garcia S, Padgett K, Moraes C. A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions. Hum Mol Genet. 2012;21:5066-77 pubmed publisher
    ..knockouts (cKOs) were created by ablation of the genes coding for the Rieske iron-sulfur protein (RISP) and COX10, respectively...
  5. Diaz F, Fukui H, Garcia S, Moraes C. Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblasts. Mol Cell Biol. 2006;26:4872-81 pubmed
    Cytochrome c oxidase (COX) biogenesis requires COX10, which encodes a protoheme:heme O farnesyl transferase that participates in the biosynthesis of heme a...
  6. Vempati U, Diaz F, Barrientos A, Narisawa S, Mian A, Millan J, et al. Role of cytochrome C in apoptosis: increased sensitivity to tumor necrosis factor alpha is associated with respiratory defects but not with lack of cytochrome C release. Mol Cell Biol. 2007;27:1771-83 pubmed
    ..These studies underscore the importance of oxidative phosphorylation and apoptosome function to both the intrinsic and extrinsic apoptotic pathways. ..
  7. Lu S, Yu Y. [Perioperative chemotherapy of stage III N2 non-small cell lung cancer.]. Zhongguo Fei Ai Za Zhi. 2008;11:652-6 pubmed publisher
  8. Fünfschilling U, Supplie L, Mahad D, Boretius S, Saab A, Edgar J, et al. Glycolytic oligodendrocytes maintain myelin and long-term axonal integrity. Nature. 2012;485:517-21 pubmed publisher
    ..Here we identify a metabolic component of axon-glia interactions by generating conditional Cox10 (protoheme IX farnesyltransferase) mutant mice, in which oligodendrocytes and Schwann cells fail to assemble ..
  9. Tarasenko T, Pacheco S, Koenig M, Gomez Rodriguez J, Kapnick S, Diaz F, et al. Cytochrome c Oxidase Activity Is a Metabolic Checkpoint that Regulates Cell Fate Decisions During T Cell Activation and Differentiation. Cell Metab. 2017;25:1254-1268.e7 pubmed publisher
    ..critical regulator of OXPHOS, we created a mouse model with isolated dysfunction in T cells by targeting a gene, COX10, that produces mitochondrial disease in humans...

More Information


  1. Diaz F, Thomas C, Garcia S, Hernandez D, Moraes C. Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency. Hum Mol Genet. 2005;14:2737-48 pubmed
    We have created a mouse model with an isolated cytochrome c oxidase (COX) deficiency by disrupting the COX10 gene in skeletal muscle. Missense mutations in COX10 have been previously associated with mitochondrial disorders...
  2. Pinto M, Nissanka N, Peralta S, Brambilla R, Diaz F, Moraes C. Pioglitazone ameliorates the phenotype of a novel Parkinson's disease mouse model by reducing neuroinflammation. Mol Neurodegener. 2016;11:25 pubmed publisher
    ..In order to analyze the role of Complex IV in PD, we knocked out Cox10 (essential for the maturation of COXI, a catalytic subunit of Complex IV) in dopaminergic neurons...
  3. Luo X, Ribeiro M, Bray E, Lee D, Yungher B, Mehta S, et al. Enhanced Transcriptional Activity and Mitochondrial Localization of STAT3 Co-induce Axon Regrowth in the Adult Central Nervous System. Cell Rep. 2016;15:398-410 pubmed publisher
  4. Tan H, Yang K, Li Y, Shaw T, Wang Y, Blanco D, et al. Integrative Proteomics and Phosphoproteomics Profiling Reveals Dynamic Signaling Networks and Bioenergetics Pathways Underlying T Cell Activation. Immunity. 2017;46:488-503 pubmed publisher
    ..Genetic perturbation revealed physiological roles for mitochondrial enzyme COX10-mediated oxidative phosphorylation in T cell quiescence exit...
  5. Supplie L, Düking T, Campbell G, Diaz F, Moraes C, Gotz M, et al. Respiration-Deficient Astrocytes Survive As Glycolytic Cells In Vivo. J Neurosci. 2017;37:4231-4242 pubmed publisher
    ..respiration in vitro and in vivo A novel mouse mutant (GLASTCreERT2::Cox10flox/flox) was generated, in which the administration of tamoxifen causes mutant astrocytes to fail ..