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SCO cytochrome oxidase deficient homolog 1

SCO1, Sco1p
Mammalian cytochrome c oxidase (COX) catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. In yeast, 2 related COX assembly genes, SCO1 and SCO2 (synthesis of cytochrome c oxidase), enable subunits 1 and 2 to be incorporated into the holoprotein. This gene is the human homolog to the yeast SCO1 gene. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: SCO2, CAN, COX17, SURF1, DR3
Papers on SCO1
COA6 is a mitochondrial complex IV assembly factor critical for biogenesis of mtDNA-encoded COX2.
Ryan et al., Melbourne, Australia. In Hum Mol Genet, Nov 2015
Furthermore, we show that COA6 has the capacity to bind copper and can associate with newly translated COX2 and the mitochondrial copper chaperone SCO1.
Aggregation of rare/low-frequency variants of the mitochondria respiratory chain-related proteins in rheumatoid arthritis patients.
Inoue et al., Isehara, Japan. In J Hum Genet, Aug 2015
An association was observed based on a pathway-burden test comprising NDUFA7, SDHAF2, SCO1 and ATP5O: P=1.56E-04, odds ratio=2.16,
The Mitochondrial Metallochaperone SCO1 Is Required to Sustain Expression of the High-Affinity Copper Transporter CTR1 and Preserve Copper Homeostasis.
Leary et al., Saskatoon, Canada. In Cell Rep, Mar 2015
UNASSIGNED: 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.
Genome-wide association mapping unravels the genetic control of seed germination and vigor in Brassica napus.
Snowdon et al., Gießen, Germany. In Front Plant Sci, 2014
These include B. napus orthologs of the Arabidopsis thaliana genes SNOWY COTYLEDON 1 (SCO1), ARABIDOPSIS TWO-COMPONENT RESPONSE REGULATOR (ARR4), and ARGINYL-t-RNA PROTEIN TRANSFERASE 1 (ATE1), which have been shown previously to play a role in seed germination and seedling growth in A. thaliana.
Establishment and characterization of an ovarian cell line from Southern catfish (Silurus meridionalis).
Wang et al., Chongqing, China. In Fish Physiol Biochem, 2014
An ovarian cell line was successfully developed from the juvenile ovary of Southern catfish (SCO1) (Silurus meridionalis), which was designated as SCO1.
D-Amino acids inhibit biofilm formation in Staphylococcus epidermidis strains from ocular infections.
Cancino-Diaz et al., Mexico. In J Med Microbiol, 2014
Recent studies have shown that d-amino acids inhibit and disrupt biofilm formation in the prototype strains Bacillus subtilis NCBI3610 and Staphylococcus aureus SCO1.
Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase.
Barrientos et al., In Hum Mol Genet, 2014
Mitochondria lacking COX20 accumulate CIV subassemblies containing COX1 and COX4, similar to those detected in fibroblasts from patients carrying mutations in the COX2 copper chaperones SCO1 and SCO2.
Divergent functions of the Arabidopsis mitochondrial SCO proteins: HCC1 is essential for COX activity while HCC2 is involved in the UV-B stress response.
Gonzalez et al., Dresden, Germany. In Front Plant Sci, 2013
The two related putative cytochrome c oxidase (COX) assembly factors HCC1 and HCC2 from Arabidopsis thaliana are Homologs of the yeast Copper Chaperones Sco1p and Sco2p.
Selective divalent copper chelation for the treatment of diabetes mellitus.
Cooper, Manchester, United Kingdom. In Curr Med Chem, 2011
These examples illustrate how impaired regulation of copper transport pathways can cause organ damage and provide important insights into the impact of defects in specific molecular processes, including those catalyzed by the copper-transporting ATPases, ATP7A (mutated in Menkes disease), ATP7B (Wilson's disease), and the copper chaperones such as those for cytochrome c oxidase, SCO1 and SCO2.
HCC1, the Arabidopsis homologue of the yeast mitochondrial copper chaperone SCO1, is essential for embryonic development.
Rödel et al., Dresden, Germany. In J Exp Bot, 2011
HCC1 of Arabidopsis is the homologue of the Saccharomyces cerevisiae SCO1.
Redox regulation of SCO protein function: controlling copper at a mitochondrial crossroad.
Leary, Saskatoon, Canada. In Antioxid Redox Signal, 2010
To date, a total of 8 soluble (COX17, COX19, COX23, PET191, CMC1-4) and 3 integral membrane (COX11, SCO1, SCO2) accessory proteins with cysteine-containing domains that reside within the mitochondrial intermembrane space (IMS) have been identified in yeast, all of which have human orthologues.
Unexpected vascular enrichment of SCO1 over SCO2 in mammalian tissues: implications for human mitochondrial disease.
Schon et al., New York City, United States. In Am J Pathol, 2010
Results describe the tissue distribution of SCO1 and SCO2 in mouse and human tissues.
Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1.
Shoubridge et al., Montréal, Canada. In Hum Mol Genet, 2009
SCO2 acts upstream of SCO1, and that it is indispensable for CO II synthesis.
Loss of function of Sco1 and its interaction with cytochrome c oxidase.
Zeman et al., Praha, Czech Republic. In Am J Physiol Cell Physiol, 2009
a fraction of Sco1 physically associates with the cytochrome c oxidase complex in human muscle mitochondria, suggesting a possible direct relationship between CcO and the regulation of cellular copper homeostasis
Novel insights into phenotype and mitochondrial proteome of yeast mutants lacking proteins Sco1p or Sco2p.
Modesti et al., Florence, Italy. In Mitochondrion, 2009
Thiese data suggest that the absence of Sco1p causes irreversible damage to the mitochondria.
Mitochondrial copper metabolism and delivery to cytochrome c oxidase.
Barrientos et al., Miami, United States. In Iubmb Life, 2008
Recent studies, mostly performed in the yeast Saccharomyces cerevisiae, have provided new clues about 1) the source of the copper used for COX metallation; 2) the roles of Sco1p and Cox11p, the proteins involved in the direct delivery of copper to the Cu(A) and Cu(B) sites, respectively; 3) the action mechanism of Cox17p, a copper chaperone that provides copper to Sco1p and Cox11p; 4) the existence of at least four Cox17p homologues carrying a similar twin CX(9)C domain suggestive of metal binding, Cox19p, Cox23p, Pet191p and Cmc1p, that could be part of the same pathway; and 5) the presence of a disulfide relay system in the intermembrane space of mitochondria that mediates import of proteins with conserved cysteines motifs such as the CX(9)C characteristic of Cox17p and its homologues.
Liver disease in mitochondrial disorders.
Sokol et al., Kuala Lumpur, Malaysia. In Semin Liver Dis, 2007
Several specific molecular defects (mutations in nuclear genes such as SCO1, BCS1L, POLG, DGUOK, and MPV17 and deletion or rearrangement of mitochondrial DNA) have been identified in recent years.
Mitochondrial hepatopathies: advances in genetics and pathogenesis.
Sokol et al., Kuala Lumpur, Malaysia. In Hepatology, 2007
In recent years, specific molecular defects (mutations in nuclear genes such as SCO1, BCS1L, POLG, DGUOK, and MPV17 and the deletion or rearrangement of mitochondrial DNA) have been identified, with the promise of genetic and prenatal diagnosis.
The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis.
Shoubridge et al., Montréal, Canada. In Cell Metab, 2007
These results suggest a mitochondrial pathway for the regulation of cellular copper content that involves signaling through SCO1 and SCO2, perhaps by their thiol redox or metal-binding state.
Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene.
Schon et al., Thessaloníki, Greece. In Nat Genet, 1999
In yeast, two related COX assembly genes, SCO1 and SCO2 (for synthesis of cytochrome c oxidase), enable subunits I and II to be incorporated into the holoprotein.
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