Table 2.

Physiological effects of associated copper chaperone proteins and their mediation in cardiac diseases.

Copper-dependent enzymes	Function	Disease consequence
CRT1	Adjust the REDOX state of copper ion; Regulate the homeostasis of copper ions within cells; High-affinity copper transfer protein	Cardiac hypertrophy; Cardiomyopathy accompanied by endocardial fibrosis and cardiac hypertrophy (59)
ATPase	ATP7A: widely expressed, except for the liver in normal conditions; regulate copper transport; regulate ATP hydrolysis rate ATP7B: existed in the liver and certain parts of the mammary tissue, kidney, placenta, and brain; regulate copper transport; regulate ATP hydrolysis rate	High prevalence of congenital heart disease
CCO	Catalyzes the final stage of respiration in cells; Electron transfer protein	Lactic acidosis; Hypertrophic cardiomyopathy
MT	Decrease oxidative stress, and apoptosis; Prevent mitochondrial morphological deterioration and reduction in creatine phosphokinase levels; Intracellular copper scavengers	Cardiac dysfunction and fibrosis (60)
SOD1	Restrain oxidative stress, autophagy and apoptosis; Catalyzes the disproportionation of superoxide to hydrogen peroxide and molecular oxygen; Oxidoreductase	Cardiac injury (inflammation and apoptosis); Early onset cardiac hypertrophy
SOD3	Decreases myocardial inflammation, fibrosis, and apoptosis	HF; Myocardial infarction; Fibrosis and IHD; Left ventricular dilation; Cardiac hypertrophy (61, 62)
CP	Negatively associated with NO; The main Cu carrier in serum; Catalyzes; Converts NO to nitrite in vivo; An oxidase for NO	Mortality; IHD; Atherosclerosis; Dyslipidemia; Obesity; DM (63)
LOX	Required for crosslinking of elastin and collagen; Converts lysine into aminoadipic semialdehyde; Oxidase	Concentric cardiac hypertrophy; Systolic dysfunction; Myocardial fibrosis (64)