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Abhishek Aich, Cong Wang, Arpita Chowdhury, Christin Ronsör, David Pacheu-Grau, Ricarda Richter-Dennerlein, Sven Dennerlein, Peter Rehling
Cytochrome c oxidase of the mitochondrial oxidative phosphorylation system reduces molecular oxygen with redox equivalent-derived electrons. The conserved mitochondrial-encoded COX1- and COX2-subunits are the heme- and copper-center containing core subunits that catalyze water formation. COX1 and COX2 initially follow independent biogenesis pathways creating assembly modules with subunit-specific, chaperone-like assembly factors that assist in redox centers formation. Here, we find that COX16, a protein required for cytochrome c oxidase assembly, interacts specifically with newly synthesized COX2 and its copper center-forming metallochaperones SCO1, SCO2, and COA6...
January 30, 2018: ELife
Trevor Carden, Bhupendra Singh, Ved Mooga, Prachi Bajpai, Keshav K Singh
The normal cellular function requires communication between mitochondria and the nucleus, termed mitochondria-to-nucleus retrograde signaling. Disruption of this mechanism has been implicated in the development of cancers. Many proteins are known modulators of retrograde signaling, but whether microRNAs (miRNAs) are also involved is unknown. We conducted an miRNA microarray analysis using RNA from a parental cell line, a Rho0 line lacking mitochondrial DNA (mtDNA) and a Rho0 line with restored mtDNA. We found that miR-663 was down-regulated in the mtDNA-depleted Rho0 line...
December 15, 2017: Journal of Biological Chemistry
Zakery N Baker, Kimberly Jett, Aren Boulet, Amzad Hossain, Paul A Cobine, Byung-Eun Kim, Amr M El Zawily, Ling Lee, Glen F Tibbits, Michael J Petris, Scot C Leary
SCO1 is a ubiquitously expressed, mitochondrial protein with essential roles in cytochrome c oxidase (COX) assembly and the regulation of copper homeostasis. SCO1 patients present with severe forms of early onset disease, and ultimately succumb from liver, heart or brain failure. However, the inherent susceptibility of these tissues to SCO1 mutations and the clinical heterogeneity observed across SCO1 pedigrees remain poorly understood phenomena. To further address this issue, we generated Sco1hrt/hrt and Sco1stm/stm mice in which Sco1 was specifically deleted in heart and striated muscle, respectively...
December 1, 2017: Human Molecular Genetics
Xiang-Bo Wei, Liang Guo, Yang Liu, Shui-Rong Zhou, Yuan Liu, Xin Dou, Shao-Yue Du, Meng Ding, Wan-Qiu Peng, Shu-Wen Qian, Hai-Yan Huang, Qi-Qun Tang
Dysregulation of insulin signaling leads to type 2 diabetes mellitus (T2DM) and other metabolic disorders. Obesity is an important contributor to insulin resistance, and although the understanding of this relationship has improved in recent years, the mechanism of obesity-induced insulin resistance is not completely understood. Disorders of copper metabolism tend to accompany the development of obesity, which increases the risk of insulin resistance. Synthesis of cytochrome c oxidase 1 (SCO1) functions in the assembly of cytochrome c oxidase (COX) and cellular copper homeostasis...
September 23, 2017: Biochemical and Biophysical Research Communications
Myriam Bourens, Antoni Barrientos
Defects in mitochondrial cytochrome c oxidase or respiratory chain complex IV (CIV) assembly are a frequent cause of human mitochondrial disorders. Specifically, mutations in four conserved assembly factors impinging the biogenesis of the mitochondrion-encoded catalytic core subunit 2 (COX2) result in myopathies. These factors afford stability of newly synthesized COX2 (the dystonia-ataxia syndrome protein COX20), a protein with two transmembrane domains, and maturation of its copper center, CuA (cardiomyopathy proteins SCO1, SCO2, and COA6)...
May 12, 2017: Journal of Biological Chemistry
Vincenza Barresi, Angela Trovato-Salinaro, Giorgia Spampinato, Nicolò Musso, Sergio Castorina, Enrico Rizzarelli, Daniele Filippo Condorelli
Copper homeostasis and distribution is strictly regulated by a network of transporters and intracellular chaperones encoded by a group of genes collectively known as copper homeostasis genes (CHGs). In this work, analysis of The Cancer Genome Atlas database for somatic point mutations in colorectal cancer revealed that inactivating mutations are absent or extremely rare in CHGs. Using oligonucleotide microarrays, we found a strong increase in mRNA levels of the membrane copper transporter 1 protein [CTR1; encoded by the solute carrier family 31 member 1 gene (SLC31A1 gene)] in our series of colorectal carcinoma samples...
August 2016: FEBS Open Bio
Hui Yang, Jia Deng, Yingying Jiang, Jiao Chen, Xianzheng Zeng, Zhiyang He, Xiaojuan Jiang, Zhuoning Li, Chunling Jiang
Emulsified isoflurane (EIso), as a result of its rapid anesthetic induction, recovery and convenience, is widely used as a novel intravenous general anesthetic. Treatment with EIso can reduce injuries caused by ischemia/reperfusion (I/R) to organs, including the heart, lung and liver, without knowing understanding the molecular mechanism. The present study hypothesized that treatment with EIso can affect the physiological processes of human lung bronchial epithelial cells (16HBE) prior to I/R. To test this hypothesis, the present study first constructed stable p53 knockdown and synthesis of cytochrome c oxidase (SCO)2 knockdown 16HBE cells...
July 2016: Molecular Medicine Reports
Petru-Iulian Trasnea, Marcel Utz, Bahia Khalfaoui-Hassani, Simon Lagies, Fevzi Daldal, Hans-Georg Koch
Copper (Cu) is an essential micronutrient that functions as a cofactor in several important enzymes, such as respiratory heme-copper oxygen reductases. Yet, Cu is also toxic and therefore cells engage a highly coordinated Cu uptake and delivery system to prevent the accumulation of toxic Cu concentrations. In this study, we analyzed Cu delivery to the cbb3 -type cytochrome c oxidase (cbb3 -Cox) of Rhodobacter capsulatus. We identified the PCuA C-like periplasmic chaperone PccA and analyzed its contribution to cbb3 -Cox assembly...
April 2016: Molecular Microbiology
Marcos N Morgada, Luciano A Abriata, Chiara Cefaro, Karolina Gajda, Lucia Banci, Alejandro J Vila
Maturation of cytochrome oxidases is a complex process requiring assembly of several subunits and adequate uptake of the metal cofactors. Two orthologous Sco proteins (Sco1 and Sco2) are essential for the correct assembly of the dicopper CuA site in the human oxidase, but their function is not fully understood. Here, we report an in vitro biochemical study that shows that Sco1 is a metallochaperone that selectively transfers Cu(I) ions based on loop recognition, whereas Sco2 is a copper-dependent thiol reductase of the cysteine ligands in the oxidase...
September 22, 2015: Proceedings of the National Academy of Sciences of the United States of America
David A Stroud, Megan J Maher, Caroline Lindau, F-Nora Vögtle, Ann E Frazier, Elliot Surgenor, Hayley Mountford, Abeer P Singh, Matteo Bonas, Silke Oeljeklaus, Bettina Warscheid, Chris Meisinger, David R Thorburn, Michael T Ryan
Biogenesis of complex IV of the mitochondrial respiratory chain requires assembly factors for subunit maturation, co-factor attachment and stabilization of intermediate assemblies. A pathogenic mutation in COA6, leading to substitution of a conserved tryptophan for a cysteine residue, results in a loss of complex IV activity and cardiomyopathy. Here, we demonstrate that the complex IV defect correlates with a severe loss in complex IV assembly in patient heart but not fibroblasts. Complete loss of COA6 activity using gene editing in HEK293T cells resulted in a profound growth defect due to complex IV deficiency, caused by impaired biogenesis of the copper-bound mitochondrial DNA-encoded subunit COX2 and subsequent accumulation of complex IV assembly intermediates...
October 1, 2015: Human Molecular Genetics
Shigeki Mitsunaga, Kazuyoshi Hosomichi, Yuko Okudaira, Hirofumi Nakaoka, Yasuo Suzuki, Masataka Kuwana, Shinji Sato, Yuko Kaneko, Yasuhiko Homma, Akira Oka, Takashi Shiina, Hidetoshi Inoko, Ituro Inoue
Exome sequencings were conducted using 59 patients having rheumatoid arthritis (RA) and 93 controls. After stepwise filtering, 107 genes showed less than 0.05 of P-values by gene-burden tests. Among 107 genes, NDUFA7 which is a subunit of the complex I in the mitochondrial respiratory chain was selected for further analysis based on previous reports. A case-control study was performed on the three single-nucleotide variants (SNVs) of NDUFA7 with 432 cases and 432 controls. An association was observed between NDUFA7 and RA with severe erosive arthritis...
August 2015: Journal of Human Genetics
Magdalena Chojnacka, Agnieszka Gornicka, Silke Oeljeklaus, Bettina Warscheid, Agnieszka Chacinska
The mitochondrial contact site and cristae organizing system (MICOS) is a recently discovered protein complex that is crucial for establishing and maintaining the proper inner membrane architecture and contacts with the outer membrane of mitochondria. The ways in which the MICOS complex is assembled and its integrity is regulated remain elusive. Here, we report a direct link between Cox17, a protein involved in the assembly of cytochrome c oxidase, and the MICOS complex. Cox17 interacts with Mic60, thereby modulating MICOS complex integrity...
June 12, 2015: Journal of Biological Chemistry
Sarah V Hatzig, Matthias Frisch, Frank Breuer, Nathalie Nesi, Sylvie Ducournau, Marie-Helene Wagner, Gunhild Leckband, Amine Abbadi, Rod J Snowdon
Rapid and uniform seed germination is a crucial prerequisite for crop establishment and high yield levels in crop production. A disclosure of genetic factors contributing to adequate seed vigor would help to further increase yield potential and stability. Here we carried out a genome-wide association study in order to define genomic regions influencing seed germination and early seedling growth in oilseed rape (Brassica napus L.). A population of 248 genetically diverse winter-type B. napus accessions was genotyped with the Brassica 60k SNP Illumina genotyping array...
2015: Frontiers in Plant Science
Leticia Martínez-Morentin, Lidia Martínez, Sarah Piloto, Hua Yang, Eric A Schon, Rafael Garesse, Rolf Bodmer, Karen Ocorr, Margarita Cervera, Juan J Arredondo
The heart is a muscle with high energy demands. Hence, most patients with mitochondrial disease produced by defects in the oxidative phosphorylation (OXPHOS) system are susceptible to cardiac involvement. The presentation of mitochondrial cardiomyopathy includes hypertrophic, dilated and left ventricular noncompaction, but the molecular mechanisms involved in cardiac impairment are unknown. One of the most frequent OXPHOS defects in humans frequently associated with cardiomyopathy is cytochrome c oxidase (COX) deficiency caused by mutations in COX assembly factors such as Sco1 and Sco2...
July 1, 2015: Human Molecular Genetics
Christopher J Hlynialuk, Binbing Ling, Zakery N Baker, Paul A Cobine, Lisa D Yu, Aren Boulet, Timothy Wai, Amzad Hossain, Amr M El Zawily, Pamela J McFie, Scot J Stone, Francisca Diaz, Carlos T Moraes, Deepa Viswanathan, Michael J Petris, Scot C Leary
Human SCO1 fulfills essential roles in cytochrome c oxidase (COX) assembly and the regulation of copper (Cu) homeostasis, yet it remains unclear why pathogenic mutations in this gene cause such clinically heterogeneous forms of disease. Here, we establish a Sco1 mouse model of human disease and show that ablation of Sco1 expression in the liver is lethal owing to severe COX and Cu deficiencies. We further demonstrate that the Cu deficiency is explained by a functional connection between SCO1 and CTR1, the high-affinity transporter that imports Cu into the cell...
February 12, 2015: Cell Reports
Miriam L Ramón-Peréz, Francisco Diaz-Cedillo, J Antonio Ibarra, Azael Torales-Cardeña, Sandra Rodríguez-Martínez, Janet Jan-Roblero, Mario E Cancino-Diaz, Juan C Cancino-Diaz
Biofilm formation on medical and surgical devices is a major virulence determinant for Staphylococcus epidermidis. The bacterium S. epidermidis is able to produce biofilms on biotic and abiotic surfaces and is the cause of ocular infection (OI). Recent studies have shown that d-amino acids inhibit and disrupt biofilm formation in the prototype strains Bacillus subtilis NCBI3610 and Staphylococcus aureus SCO1. The effect of d-amino acids on S. epidermidis biofilm formation has yet to be tested for clinical or commensal isolates...
October 2014: Journal of Medical Microbiology
Jing Wei, WenChuang Qi, Yujie Zhou, Xiaoping Zhang, Ranran Dong, Linyan Zhou, Deshou Wang
An ovarian cell line was successfully developed from the juvenile ovary of Southern catfish (SCO1) (Silurus meridionalis), which was designated as SCO1. The cell line multiplied preferentially in L-15 medium with 15 % fetal bovine serum at 28 °C for more than 70 passages over a period of 420 days. SCO1 showed fibroblast-like morphology and predominantly retained a diploid karyotype of 58 chromosomes. From the gene expression patterns, SCO1 showed a characteristic of ovarian granulosa cells. After the cells were transfected with the green fluorescent protein expression vector, bright fluorescent signals could be observed in approximately 30 % cells...
October 2014: Fish Physiology and Biochemistry
Myriam Bourens, Aren Boulet, Scot C Leary, Antoni Barrientos
Cytochrome c oxidase (CIV) deficiency is one of the most common respiratory chain defects in patients presenting with mitochondrial encephalocardiomyopathies. CIV biogenesis is complicated by the dual genetic origin of its structural subunits, and assembly of a functional holoenzyme complex requires a large number of nucleus-encoded assembly factors. In general, the functions of these assembly factors remain poorly understood, and mechanistic investigations of human CIV biogenesis have been limited by the availability of model cell lines...
June 1, 2014: Human Molecular Genetics
Arnaud Mourier, Benedetta Ruzzenente, Tobias Brandt, Werner Kühlbrandt, Nils-Göran Larsson
Defects of the oxidative phosphorylation system, in particular of cytochrome-c oxidase (COX, respiratory chain complex IV), are common causes of Leigh syndrome (LS), which is a rare neurodegenerative disorder with severe progressive neurological symptoms that usually present during infancy or early childhood. The COX-deficient form of LS is commonly caused by mutations in genes encoding COX assembly factors, e.g. SURF1, SCO1, SCO2 or COX10. However, other mutations affecting genes that encode proteins not directly involved in COX assembly can also cause LS...
May 15, 2014: Human Molecular Genetics
Andrew M Thomas, Bo-Lin Lin, Erik C Wasinger, T Daniel P Stack
Copper thiolate/disulfide interconversions are related to the functions of several important proteins such as human Sco1, Cu-Zn superoxide dismutase (SOD1), and mammalian zinc-bonded metallothionein. The synthesis and characterization of well-defined synthetic analogues for such interconversions are challenging yet provide important insights into the mechanisms of such redox processes. Solvent-dependent redox isomerization and proton-coupled electron transfer mimicking these interconversions are observed in two structurally related dimeric μ,η(2):η(2)-thiolato Cu(II)Cu(II) complexes by various methods, including X-ray diffraction, XAS, NMR, and UV-vis...
December 18, 2013: Journal of the American Chemical Society
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