. Author manuscript; available in PMC: 2024 Jan 1.

Published in final edited form as: Trends Endocrinol Metab. 2022 Nov 23;34(1):21–33. doi: 10.1016/j.tem.2022.11.001

Table 1.

Genes involved in mitochondrial copper biology.

Yeast Gene	Human Gene	Function	Refs
COX1	COX1	Subunit of the catalytic core of CcO, contains the Cu_B site, catalyzes electron transport to molecular oxygen	[24]
COX2	COX2	Subunit of the catalytic core of CcO, contains the Cu_A site, catalyzes electron transport to molecular oxygen	[24]
COX11	COX11	Metallochaperone, a component of the copper delivery pathway to the Cu_B site	[38, 39]
COX17	COX17	Metallochaperone, transfers copper to metallochaperones SCO1 and COX11	[36]
COX19	COX19	Precise function unknown, a component of the copper delivery pathway to the Cu_B site	[46]
COA4	COA4	Precise function unknown, a component of the copper delivery pathway to the CuB site	[45]
COA6	COA6	Disulfide reductase of COX2 and SCO1, a component of the copper delivery pathway to the Cu_A site	[41, 42, 97]
SCO1	SCO1	Metallochaperone, required for copper delivery to the Cu_A site	[37]
SCO2	SCO2	Copper-binding thiol-disulfide oxidoreductase of COX2, a component of the copper delivery pathway to the Cu_A site	[37, 40]
PIC2	SLC25A3	Mitochondrial copper importer	[28, 29]
CCS1	CCS	Metallochaperone, transfers copper to SOD1	[16]
SOD1	SOD1	Cytosol and mitochondria localized copper-zinc superoxide dismutase, detoxifies superoxides	[16, 98]