Recombinant Human OPA3 protein

Supplementary information

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

OPA3 also known as Optic Atrophy 3 or Optic atrophy protein 3 is a protein with a mass of approximately 36 kDa. This protein is mainly present in the mitochondria which are responsible for energy production in cells. It plays an essential mechanical role in maintaining mitochondrial integrity. The gene encoding OPA3 expresses in various tissues with notable levels in the brain skeletal muscle and to some extent in other organs like the liver and kidney.

Biological function summary

The role of OPA3 is significant due to its involvement in the regulation of mitochondrial dynamics. It helps balance mitochondrial fusion and fission processes that ensure proper mitochondrial function and energy production. OPA3 participates in a complex that includes several other mitochondrial proteins necessary for preserving mitochondrial structure and function. This complex contributes to the regulation of metabolic pathways that are important for normal cellular function.

Pathways

The involvement of OPA3 in mitochondrial dynamics links it closely to the mitochondrial fission and fusion pathways. These pathways are critical for mitochondrial quality control which affects cellular energy homeostasis and apoptosis. Proteins like MFN1 and DRP1 interact with or relate to OPA3 as they play roles in the same pathways contributing to maintaining mitochondrial health and preventing cellular stress.

The impairment of OPA3 function associates with conditions such as autosomal dominant optic atrophy and Costeff syndrome. Optic atrophy linked with OPA3 leads to degeneration in optic nerve fibers and consequent vision loss. Costeff syndrome a disorder that affects multiple body systems also involves defects in OPA3. Such conditions highlight connections with proteins like MFN2 known for involvement in Charcot-Marie-Tooth disease underlining the importance of mitochondrial health to prevent neural and optic disorders.