The SURF1 gene provides instructions for making a protein that is important in oxidative phosphorylation, the process by which the energy from food is converted into a form cells can use. Oxidative phosphorylation involves a series of reactions that take place through several different protein complexes. The SURF1 protein aids in the correct assembly of one of the protein complexes, or enzymes, involved in oxidative phosphorylation called complex IV.
Complex IV, also known as cytochrome c oxidase or COX, accepts negatively charged particles (electrons) from earlier steps in oxidative phosphorylation. In addition, the enzyme accepts positively charged particles (protons) from inside the mitochondrion. Using the electrons and protons, the COX enzyme performs a chemical reaction that converts oxygen to water. The enzyme also transfers additional protons across the specialized membrane inside the mitochondrion. These processes create energy that is used to generate adenosine triphosphate (ATP), the cell’s main energy source.
Other Names
SURF-1; SURF1_HUMAN; surfeit 1; surfeit locus protein 1
Related Genetics Topics
- Charcot-Marie-Tooth disease — MedlinePlus Genetics
- Leigh syndrome — MedlinePlus Genetics
- Cytochrome c oxidase deficiency — MedlinePlus Genetics
Identifiers
- NCBI Gene: 6834
- OMIM: 185620
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
