Gene Symbol: COX6A1
Description: cytochrome c oxidase subunit 6A1
Alias: CMTRID, COX6A, COX6AL, cytochrome c oxidase subunit 6A1, mitochondrial, COX VIa-L, cytochrome C oxidase subunit VIa homolog, cytochrome c oxidase polypeptide VIa-liver, cytochrome c oxidase subunit VIA-liver, cytochrome c oxidase subunit VIa polypeptide 1
- Mechanism of super-assembly of respiratory complexes III and IVSara Cogliati
Centro Nacional de Investigaciones Cardiovasculares Carlos III, 28029 Madrid, Spain
Nature 539:579-582. 2016..Furthermore, we find that the CIV subunit COX7A2 is replaced by SCAF1 in supercomplexes containing CIII and CIV and by COX7A1 in CIV dimers, and that dimers seem to be more stable when they include COX6A2 rather than the COX6A1 isoform.
- mRNA degradation: an underestimated factor in steady-state transcript levels of cytochrome c oxidase subunits?Katharina Bremer
Department of Biology, Queen s University, Kingston, ON K7L 3N6, Canada
J Exp Biol 217:2212-20. 2014..not explain the patterns seen with another subunit that did not change in mRNA level with thermal acclimation (COX6A-2)...
- A mutation of COX6A1 causes a recessive axonal or mixed form of Charcot-Marie-Tooth diseaseGen Tamiya
Advanced Molecular Epidemiology Research Institute, Faculty of Medicine, Yamagata University, Yamagata 990 9585, Japan Electronic address
Am J Hum Genet 95:294-300. 2014..in a splicing element (pyrimidine tract) of intron 2 adjacent to the third exon of cytochrome c oxidase subunit VIa polypeptide 1 (COX6A1), which is a component of mitochondrial respiratory complex IV (cytochrome c oxidase [..
- Comparison of PBDE congeners as inducers of oxidative stress in zebrafishCrystal Y Usenko
Department of Biology, Baylor University, Waco, Texas, USA
Environ Toxicol Chem 34:1154-60. 2015..At 24 hpf, cytochrome c oxidase subunit 6a (COX6a) transcription was up-regulated in embryos exposed to BDE 99, and BDE 28 exposure up-regulated the transcription ..
- Investigating the role of the physiological isoform switch of cytochrome c oxidase subunits in reversible mitochondrial diseaseVeronika Boczonadi
Institute of Genetic Medicine, Wellcome Trust Mitochondrial Research Centre, Newcastle University, Central Parkway NE1 3BZ Newcastle upon Tyne, UK
Int J Biochem Cell Biol 63:32-40. 2015..Isoforms of subunits COX6A and COX7A expressed in heart and skeletal muscle are different from isoforms expressed in the liver, kidney and ..
- Transcriptome markers of viral persistence in naturally-infected andes virus (bunyaviridae) seropositive long-tailed pygmy rice ratsCorey L Campbell
Arthropod Borne and Infectious Diseases Laboratory, Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, United States of America
PLoS ONE 10:e0122935. 2015..In the remaining acutely infected rice rat, the apoptotic pathway marker Cox6a1 was elevated, and putative anti-viral factors Abcb1a, Fam46c, Spp1, Rxra, Rxrb, Trmp2 and Trim58 were modulated...
- ALS5/SPG11/KIAA1840 mutations cause autosomal recessive axonal Charcot-Marie-Tooth diseaseCeleste Montecchiani
1 Laboratorio di Neurogenetica, CERC IRCCS Santa Lucia, Rome, Italy
Brain 139:73-85. 2016..CMT2B5/NEFL, ARCMT2F/dHMN2B/HSPB1, CMT2K/GDAP1, CMT2P/LRSAM1, CMT2R/TRIM2, CMT2S/IGHMBP2, CMT2T/HSJ1, CMTRID/COX6A1, ARAN-NM/HINT and GAN/GAN), for the genes related to autosomal recessive hereditary spastic paraplegia with ..
- The mammalian homologue of yeast Afg1 ATPase (lactation elevated 1) mediates degradation of nuclear-encoded complex IV subunitsJana Cesnekova
Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague, Prague, Czech Republic
Biochem J 473:797-804. 2016..that LACE1 mediates degradation of nuclear-encoded complex IV subunits COX4 (cytochrome c oxidase 4), COX5A and COX6A, and is required for normal activity of complexes III and IV of the respiratory chain...
- Iron Deficiency Impairs Developing Hippocampal Neuron Gene Expression, Energy Metabolism, and Dendrite ComplexityThomas W Bastian
Department of Pediatrics and Center for Neurobehavioral Development, School of Medicine, Minneapolis, Minn, USA
Dev Neurosci 38:264-276. 2016..e. BdnfVI,Camk2a,Vamp1,Psd95,Cfl1, Pfn1,Pfn2, and Gda) and mitochondrial function (i.e. Ucp2,Pink1, and Cox6a1)...
- Functional analysis of the rat cytochrome c oxidase subunit 6A1 promoter in primary neuronsSakkapol Ongwijitwat
Department of Cell Biology, Neurobiology, and Anatomy, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, USA
Gene 337:163-71. 2004..In vivo binding of NRF-2 to the rCOX6A1 promoter was confirmed with chromatin immunoprecipitation assay (ChIP). We conclude that Sp1, NRF-1, and NRF-2 are important in activating transcription of the rat COX6A1 gene.
- DNA microarray and miRNA analyses reinforce the classification of follicular thyroid tumorsCaroline Jacques
Institut National de la Santé et de la Recherche Médicale U694, rue des Capucins, F 49033 Angers, France
J Clin Endocrinol Metab 98:E981-9. 2013..Focusing on mitochondrial function and thyroid tumorigenesis, we used an integrative approach to identify relevant biomarkers for borderline thyroid lesions...
- High-resolution melting analysis of 15 genes in 60 patients with cytochrome-c oxidase deficiencyAlzbeta Vondrácková
Department of Pediatrics and Adolescent Medicine, Charles University in Prague and General University Hospital in Prague, Prague, Czech Republic
J Hum Genet 57:442-8. 2012..Nine novel variants were identified in exons and adjacent intronic regions of COX4I2, COX6A1, COX6A2, COX7A1, COX7A2 and COX10 using high-resolution melting (HRM) analysis...
- HIV-1 Tat protein directly induces mitochondrial membrane permeabilization and inactivates cytochrome c oxidaseH Lecoeur
THERAPTOSIS SA, Pasteur BioTop Research Laboratory, Institut Pasteur, Paris, France
Cell Death Dis 3:e282. 2012....
- Evolution of the nuclear-encoded cytochrome oxidase subunits in vertebratesA G Little
Department of Biology, Queen s University, Kingston, Canada
Physiol Genomics 42:76-84. 2010..were identical throughout the major vertebrate taxa: no paralogs of COX6c and COX7c, two paralogs of COX4 and COX6a, and three paralogs of COX7a...
- Selective lamina dysregulation in granular retrosplenial cortex (area 29) after anterior thalamic lesions: an in situ hybridization and trans-neuronal tracing study in ratsE Amin
School of Psychology, Cardiff University, Wales CF10 3AT, UK
Neuroscience 169:1255-67. 2010..Three other markers (cox6a1, CD74, ncs-1) did not appear to change activity levels after surgery...
- Polymorphic variation in cytochrome oxidase subunit genesJianghua Lu
Department of Neurology, University of Kansas School of Medicine, Kansas City, KS 66160, USA
J Alzheimers Dis 21:141-54. 2010..Frequent untranslated region (UTR) polymorphisms were seen in COX6A1, COX6B1, COX6C, and COX7A1; heterogeneity in a COX7A1 5' UTR Sp1 site was extensive...
- Novel insights into the assembly and function of human nuclear-encoded cytochrome c oxidase subunits 4, 5a, 6a, 7a and 7bDaniela Fornuskova
Department of Pediatrics, First Faculty of Medicine, Charles University in Prague, Ke Karlovu 2, Prague 2, 12808 Czech Republic
Biochem J 428:363-74. 2010..CcO subunits in the assembly and function of the human complex, we used stable RNA interference of COX4, COX5A and COX6A1, as well as expression of epitope-tagged Cox6a, Cox7a and Cox7b, in HEK (human embryonic kidney)-293 cells...
- Differential expression of genes specifying two isoforms of subunit VIa of human cytochrome c oxidaseG M Fabrizi
H Houston Merritt Clinical Research Center for Muscular Dystrophy and Related Disorders, Columbia University College of Physicians and Surgeons, New York, NY 10032
Gene 119:307-12. 1992..The cDNA specifying COX VIa-M is a prime candidate for use in investigations of Mendelian-inherited COX deficiencies with primary involvement of muscle...
- Identification of microRNAs regulated by activin A in human embryonic stem cellsZong Yun Tsai
Department of Medicinal and Applied Chemistry, College of Life Sciences, Kaohsiung Medical University, Kaohsiung 807, Taiwan
J Cell Biochem 109:93-102. 2010..Thirteen abundantly differentially expressed mRNAs, including NR4A2, ERBB4, CXCR4, PCDH9, TMEFF2, CD24, and COX6A1 genes, targeted by seven over-expressed miRNAs were identified by inverse expression levels of these seven ..
- Assembly of nuclear DNA-encoded subunits into mitochondrial complex IV, and their preferential integration into supercomplex forms in patient mitochondriaMichael Lazarou
Department of Biochemistry, La Trobe University, Melbourne, Australia
FEBS J 276:6701-13. 2009..Using in vitro import and assembly assays, we found that subunits Cox6a, Cox6b and Cox7a assembled into pre-existing complex IV, while Cox4-1 and Cox6c subunits assembled into ..
- Expression of the peptide hormone hepcidin increases in cardiomyocytes under myocarditis and myocardial infarctionManabu Isoda
Department of Thoracic and Cardiovascular Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
J Nutr Biochem 21:749-56. 2010..Analysis of the time course of the hepcidin to cytochrome oxidase subunit 6a (Cox6a)2 expression ratio showed that it abruptly increased more than 100-fold in hearts in the very early phase of EAM ..
- Drosophila RNAi screen identifies host genes important for influenza virus replicationLinhui Hao
Institute for Molecular Virology, USA
Nature 454:890-3. 2008..genes identified; that is, for three implicated Drosophila genes, the corresponding human homologues ATP6V0D1, COX6A1 and NXF1 are shown to have key functions in the replication of H5N1 and H1N1 influenza A viruses, but not ..
- Identification of cytochrome c oxidase subunit 6A1 as a suppressor of Bax-induced cell death by yeast-based functional screeningSo Young Eun
Department of Pharmacology, Gyeongsang Institute of Health Science, Gyeongsang National University School of Medicine, 92 Chilam dong, Jinju 660 751, Republic of Korea
Biochem Biophys Res Commun 373:58-63. 2008Human cytochrome c oxidase subunit VIa polypeptide 1 (COX6A1) was identified as a novel suppressor of Bcl-2-associated X protein (Bax)-mediated cell death using yeast-based functional screening of a mammalian cDNA library...
- Nuclear respiratory factor 1 controls myocyte enhancer factor 2A transcription to provide a mechanism for coordinate expression of respiratory chain subunitsBindu Ramachandran
Diabetes Research Laboratory, Department of Medicine, Massachusetts General Hospital, Charlestown, Massachusetts 02129, USA
J Biol Chem 283:11935-46. 2008..We used genome comparisons to conclude that the promoter regions of COX6A(H) and COX7A(H) lack NRF sites but have conserved myocyte enhancer factor 2 (MEF2) elements...
- Human COX6A1 gene: promoter analysis, cDNA isolation and expression in the monkey brainM Wong-Riley
Department of Cell Biology, Neurobiology and Anatomy, Medical College of Wisconsin, Milwaukee, WI 53226, USA
Gene 247:63-75. 2000The human COX6A1 gene encodes the ubiquitous isoform of cytochrome c oxidase (COX) subunit VIa (VIa-L), and is located in a CpG island on chromosome 12q24.2...
- Assignment of COX6A1 to 6p21 and a pseudogene (COX6A1P) to 1p31.1 by in situ hybridization and somatic cell hybridsY Hey
CRC Department of Cancer Genetics, Paterson Institute for Cancer Research, Manchester, UK
Cytogenet Cell Genet 77:167-8. 1997
- Cloning, characterization, and chromosomal localization of human liver form cytochrome c oxidase subunit VIa related genesF Merante
Centre for Cardiovascular Research, Toronto Hospital, ON, Canada
Genome 40:325-31. 1997..In total, four COX VIa-L related sequences have been cloned and their nucleotide sequences analyzed. At least three of the sequences represent pseudogenes; their relatedness to the COX VIa-L cDNA is discussed...
- Molecular evolution of cytochrome c oxidase: rate variation among subunit VIa isoformsT R Schmidt
Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, Michigan 48201, USA
Mol Biol Evol 14:595-601. 1997..acid replacement) substitutions in the COX6AH gene occurred more frequently than in the ubiquitously expressed COX6AL gene...
- Tissue distribution of cytochrome c oxidase isoforms in mammals. Characterization with monoclonal and polyclonal antibodiesJ W Taanman
Institute of Molecular Biology, University of Oregon, Eugene 97403
Biochim Biophys Acta 1225:95-100. 1993..These results imply that, in humans, the deficiency of a subunit VIa isoform may have a different effect on the physiology of heart then on the physiology of skeletal muscle...
- Cloning, sequence analysis, and expression of a mouse cDNA encoding cytochrome c oxidase subunit VIa liver isoformL I Grossman
Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, MI 48201
Biochim Biophys Acta 1260:361-4. 1995A cDNA encoding cytochrome c oxidase (COX) subunit VIa liver isoform (COX6aL) was isolated from a Mus musculus library and sequenced...
- Structural organization of the bovine gene for the heart/muscle isoform of cytochrome c oxidase subunit VIaE O Smith
Department of Anatomy and Cell Biology, University of Michigan, Ann Arbor 48109 0616
Biochim Biophys Acta 1174:63-71. 1993The bovine gene for the nuclear-encoded heart/muscle isoform of cytochrome c oxidase subunit VIa (COX6A1) was isolated from a library of bovine genomic DNA in lambda EMBL3 and sequenced...
- Sequence of a cDNA specifying subunit VIa of human cytochrome c oxidaseG M Fabrizi
Department of Neurology, Columbia University College of Physicians and Surgeons, New York, NY 10032
Nucleic Acids Res 17:6409. 1989
- METABOLIC ORGANIZATION & REGULATION IN THE VISUAL CORTEXMargaret Wong Riley; Fiscal Year: 2002..Results from these studies are expected to provide insight into the molecular basis of metabolic responses of visual cortical neurons to changing functional demands. ..
- Neurochemical Organization of Brain Stem Respiratory NucleiMARGARET TT WONG RILEY; Fiscal Year: 2010....