Copper is an important trace element that is required for essential enzymes. However, due
to its redox activity, copper can also lead to the generation of toxic reactive oxygen species …

The current state of knowledge on how copper metallochaperones support the maturation of
cuproproteins is reviewed. Copper is needed within mitochondria to supply the CuA and …

Copper (Cu) is an essential micronutrient required for the activity of redox‐active enzymes
involved in critical metabolic reactions, signaling pathways, and biological functions …

Throughout evolution, all organisms have harnessed the redox properties of copper (Cu)
and iron (Fe) as a cofactor or structural determinant of proteins that perform critical functions …

Eukaryotic cytochrome c oxidase (COX) is the terminal enzyme of the mitochondrial
respiratory chain. COX is a multimeric enzyme formed by subunits of dual genetic origin …

Copper plays an indispensable role in the physiology of the human central nervous system
(CNS). As a cofactor of dopamine-β-hydroxylase, peptidyl-α-monooxygenase, superoxide …

Mitochondria are double-membrane organelles that contain their own genome, the
mitochondrial DNA (mtDNA), and reminiscent of its endosymbiotic origin. Mitochondria are …

Copper is a biometal essential for normal brain development and function, thus copper
deficiency or excess results in central nervous system disease. Well-characterized disorders …

Biogenesis of complex IV of the mitochondrial respiratory chain requires assembly factors for
subunit maturation, co-factor attachment and stabilization of intermediate assemblies. A …

Accumulation of mitochondrial electron transport chain (ETC) defects is a recognized
hallmark of the age-associated decline in cardiac bioenergetics; however, the molecular …