About SURF / Surfeit:
Surfeit locus protein 1 (SURF1) is a multi-pass protein believed to be used in the mitochondrial translation regulation assembly intermediate of cytochrome c oxidase complexes (also known as Complex IV, COX, and/or MITRAC complex). It thus plays a role in the regulation of the cytochrome c oxidase assembly.
Mutations in SURF1 are strongly linked with Leigh syndrome. This in turn is linked with systemic cytochrome c oxidase (complex IV) deficiency, and Charcot-Marie-Tooth disease 4K (CMT4K).
The SURF1 protein is found in the inner mitochondrial membrane. It is believed to play a role in the biogenesis of Complex IV. Complex IV plays a vital role in oxidative phosphorylation. It attracts electrons created through previous steps in oxidative phosphorylation and protons from inside the mitochondrion. Using these, it converts oxygen to water.
Additionally, Complex IV sends protons across the specialized membrane inside the mitochondrion. This creates energy which is then converted into adenosine triphosphate (ATP). This is the form of energy mainly used for cellular processes.
The SURF1 gene forms part of the surfeit gene cluster. These genes are very tightly linked but do not have similar sequences. The SURF1 gene is located in position 34.2 of the q arm of chromosome 9 (9q34.2). It has 9 exons. The SURF1 protein is a 33.3 kDa protein. It consists of 300 amino acids.
SURF1 has a total of 11 binary protein-protein interactions. Of these, 8 are co-complex interactions. Genes known to interact with SURF1 include: