SOD1:Zn2+ apoenzyme binds CCS:Zn2+:2xCu1+

Stable Identifier
Homo sapiens
Locations in the PathwayBrowser

Copper chaperone of superoxide dismutase (CCS) transfers a copper(I) atom to a SOD1 monomer that already contains a Zn atom (Culotta et al. 1997, Casareno et al. 1998, Rae et al. 2001, Brown et al. 2004, Banci et al. 2012). The reaction proceeds by a two step mechanism in which SOD1 first forms heterodimers with CCS (Rae et al. 2001, Banci et al. 2012).

Literature References
PubMed ID Title Journal Year
9726962 The copper chaperone CCS directly interacts with copper/zinc superoxide dismutase

Casareno, RL, Waggoner, D, Gitlin, JD

J. Biol. Chem. 1998
9295278 The copper chaperone for superoxide dismutase

Culotta, VC, Klomp, LW, Strain, J, Casareno, RL, Krems, B, Gitlin, JD

J. Biol. Chem. 1997
15064408 Oxygen and the copper chaperone CCS regulate posttranslational activation of Cu,Zn superoxide dismutase

Brown, NM, Torres, AS, Doan, PE, O'Halloran, TV

Proc. Natl. Acad. Sci. U.S.A. 2004
22869735 Human superoxide dismutase 1 (hSOD1) maturation through interaction with human copper chaperone for SOD1 (hCCS)

Banci, L, Bertini, I, Cantini, F, Kozyreva, T, Massagni, C, Palumaa, P, Rubino, JT, Zovo, K

Proc. Natl. Acad. Sci. U.S.A. 2012
16880213 The effects of glutaredoxin and copper activation pathways on the disulfide and stability of Cu,Zn superoxide dismutase

Carroll, MC, Outten, CE, Proescher, JB, Rosenfeld, L, Watson, WH, Whitson, LJ, Hart, PJ, Jensen, LT, Cizewski Culotta, V

J. Biol. Chem. 2006
18703498 Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria

Kawamata, H, Manfredi, G

Hum. Mol. Genet. 2008
11018045 Mechanism of Cu,Zn-superoxide dismutase activation by the human metallochaperone hCCS

Rae, TD, Torres, AS, Pufahl, RA, O'Halloran, TV

J. Biol. Chem. 2001
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Orthologous Events