Recombinant Human Protein SCO1 homolog, mitochondrial (SCO1)

Uniprot Id

O75880

Target Species

Human

Target Name

SCO1

Target Full Name

Protein SCO1 homolog, mitochondrial

Target Function

Copper metallochaperone essential for the maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Not required for the synthesis of MT-CO2/COX2 but plays a crucial role in stabilizing MT-CO2/COX2 during its subsequent maturation. Involved in transporting copper to the Cu(A) site on MT-CO2/COX2. Plays an important role in the regulation of copper homeostasis by controlling the abundance and cell membrane localization of copper transporter CTR1.

Target Involvement

Mitochondrial complex IV deficiency (MT-C4D)

Target Subcellular Location

Mitochondrion. Mitochondrion inner membrane; Single-pass membrane protein.

Target Protein Families

SCO1/2 family

Target Tissue Specificity

Predominantly expressed in tissues characterized by high rates of oxidative phosphorylation (OxPhos), including muscle, heart, and brain.

Target Synonyms

Cytochrome oxidase deficient homolog; Cytochrome oxidase deficient homolog 1; Protein SCO1 homolog mitochondrial; Protein SCO1 homolog, mitochondrial; SCO (cytochrome oxidase deficient yeast) homolog 1; SCO cytochrome oxidase deficient homolog 1 (yeast); SCO cytochrome oxidase deficient homolog 1; sco1; SCO1_HUMAN; SCOD1

Target Background

Mammalian cytochrome c oxidase (COX) catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. In yeast, 2 related COX assembly genes, SCO1 and SCO2 (synthesis of cytochrome c oxidase), enable subunits 1 and 2 to be incorporated into the holoprotein. This gene is the human homolog to the yeast SCO1 gene.