Anti-SCO2 / Copper chaperone SCO2

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. SCO2 antibody detects the Copper chaperone SCO2, a mitochondrial inner membrane protein required for the assembly of cytochrome c oxidase (complex IV) of the respiratory chain. The SCO2 gene encodes a metallochaperone that facilitates copper delivery to the COX2 subunit of cytochrome c oxidase, ensuring proper enzyme maturation and electron transport. SCO2 plays an essential role in aerobic energy metabolism, and its deficiency leads to mitochondrial dysfunction and severe metabolic disorders.Located on chromosome 22q13.33, SCO2 (Synthesis of Cytochrome c Oxidase 2) is one of two homologous human SCO genes, the other being SCO1. Both are nuclear-encoded but localized to mitochondria, where they coordinate copper homeostasis. SCO2 binds copper ions via conserved cysteine and histidine residues and transfers them to cytochrome oxidase subunits through direct protein-protein interactions. Mutations in SCO2 disrupt this process and result in cytochrome c oxidase deficiency, leading to diseases such as fatal infantile cardioencephalomyopathy and Leigh-like syndrome.SCO2 expression is regulated by hypoxia and cellular copper status. Under low oxygen conditions, transcription factor HIF-1 may influence SCO2 expression to modulate oxidative phosphorylation efficiency. The protein is also involved in maintaining redox balance and preventing the accumulation of reactive oxygen species (ROS) within mitochondria. Beyond its role in energy production, SCO2 contributes to cellular copper distribution and may participate in signaling pathways that coordinate mitochondrial biogenesis.Immunohistochemical analysis using SCO2 antibody demonstrates mitochondrial localization in heart, skeletal muscle, and brain tissues, consistent with its role in energy metabolism. The antibody serves as a key tool for studying mitochondrial function, copper metabolism, and respiratory chain biogenesis. SCO2 antibody from NSJ Bioreagents provides reliable detection of this essential metallochaperone in biochemical and cell biology research applications.