SCO Cytochrome Oxidase Deficient Homolog 1 (SCO1)

The sco1 protein is a constituent of the inner mitochondrial membrane and is thought to play a role in either mitochondrial copper transport or insertion of copper into the active site of COX.

The predicted 301-amino acid human protein shares 34% identity with yeast sco1. SCOD1 contains a mitochondrial leader peptide, a putative transmembrane domain, and a copper-binding domain. In vitro mitochondrial import and trypsin-protection assays demonstrated that the SCOD1 protein is targeted to mitochondria. Northern blot analysis revealed that SCOD1 is predominantly expressed in tissues characterized by high rates of oxidative phosphorylation (OXPHOS), including muscle, heart, and brain.