scholarly journals A genome-wide copper-sensitized screen identifies novel regulators of mitochondrial cytochrome c oxidase activity

2021 ◽  
Vol 296 ◽  
pp. 100485
Author(s):  
Natalie M. Garza ◽  
Aaron T. Griffin ◽  
Mohammad Zulkifli ◽  
Chenxi Qiu ◽  
Craig D. Kaplan ◽  
...  
2011 ◽  
Vol 33 (1) ◽  
pp. 50-55 ◽  
Author(s):  
Mandy L. Marziaz ◽  
Kathryn Frazier ◽  
Paul B. Guidry ◽  
Robyn A. Ruiz ◽  
Ilona Petrikovics ◽  
...  

2002 ◽  
Vol 363 (2) ◽  
pp. 321-327 ◽  
Author(s):  
Leonardo SALVIATI ◽  
Evelyn HERNANDEZ-ROSA ◽  
Winsome F. WALKER ◽  
Sabrina SACCONI ◽  
Salvatore DiMAURO ◽  
...  

Human SCO2 is a nuclear-encoded Cu-binding protein, presumed to be responsible for the insertion of Cu into the mitochondrial cytochrome c oxidase (COX) holoenzyme. Mutations in SCO2 are associated with cardioencephalomyopathy and COX deficiency. Studies in yeast and bacteria have shown that Cu supplementation can restore COX activity in cells harbouring mutations in genes involving Cu transport. Therefore we investigated whether Cu supplementation could restore COX activity in cultured cells from patients with SCO2 mutations. Our data demonstrate that the COX deficiency observed in fibroblasts, myoblasts and myotubes from patients with SCO2 mutations can be restored to almost normal levels by the addition of CuCl2 to the growth medium.


2021 ◽  
Vol 271 ◽  
pp. 116377
Author(s):  
Libing Yu ◽  
Wenjing Li ◽  
Jian Chu ◽  
Chun Chen ◽  
Xijian Li ◽  
...  

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