Anti-Surf1抗体

补充信息

This supplementary information is collated from multiple sources and compiled automatically.

Surf1 also known as SURF1 homolog or SURF1 cytochrome c oxidase assembly protein has a molecular mass of approximately 33 kDa. It localizes mainly in the inner mitochondrial membrane of eukaryotic cells. The expression of Surf1 is widespread found in tissues with high metabolic demand like the heart brain and skeletal muscle. Surf1 plays an important role in the proper functioning of mitochondria which are the powerhouses of the cell generating energy in the form of ATP.

Biological function summary

Surf1 assists in the assembly of cytochrome c oxidase (COX) the last enzyme in the mitochondrial respiratory chain complex IV. It interacts with various subunits of the COX complex ensuring their proper assembly and stability within the inner mitochondrial membrane. Through its role in COX assembly Surf1 supports efficient electron transport and energy production which is critical for cellular respiration.

Pathways

Surf1 contributes to electron transport chain pathways and oxidative phosphorylation. It facilitates the integration of COX subunits into a functional complex. Surf1 interacts with other mitochondrial proteins like COX10 and COX15 which are involved in heme A biosynthesis a critical component of COX. Together these proteins ensure the efficient production and transfer of ATP maintaining cellular energy balance.

Surf1 mutations relate strongly to Leigh syndrome a severe neurological disorder. This disorder results from defective energy production due to impaired cytochrome c oxidase assembly. Children with Leigh syndrome often exhibit symptoms like movement disorders and brain abnormalities. Surf1 abnormalities can also connect with other conditions involving compromised mitochondrial function linking it to proteins involved in mitochondrial maintenance and function such as NDUFS4. These associations emphasize the significance of Surf1 in maintaining cellular health and energy production.