SURF1 Human

Surfeit-1 is a single, non-glycosylated polypeptide chain containing 215 amino acids, with a molecular mass of approximately 24.3 kDa . The recombinant form of this protein is often produced in E. coli and is fused to a 20 amino acid His Tag at the N-terminus for purification purposes . The protein is typically purified using proprietary chromatographic techniques .

Surfeit-1 is localized to the inner mitochondrial membrane and is thought to play a crucial role in the biogenesis of the cytochrome c oxidase complex . This complex is essential for the mitochondrial electron transport chain, which is critical for cellular respiration and energy production.

Mutations in the SURF1 gene are associated with Leigh syndrome, a severe neurological disorder characterized by systemic cytochrome c oxidase deficiency . This condition leads to progressive loss of mental and movement abilities, and often results in early childhood death.

Recombinant Surfeit-1 is stable at 4°C for up to 4 weeks, but for long-term storage, it should be kept desiccated below -18°C . It is recommended to add a carrier protein, such as 0.1% HSA or BSA, to prevent freeze-thaw cycles .

Surfeit-1 (Human Recombinant) is primarily used in research settings to study mitochondrial function and disorders related to cytochrome c oxidase deficiency. It is also used in various biochemical assays and structural studies to understand its role in mitochondrial biogenesis.