OPA3, also known as optic atrophy 3, is a key mitochondrial membrane protein [1]. It exists in mitochondria, the energy production center of cells, and is involved in multiple biological processes. Mutations in OPA3 can lead to 3-methylglutaconic aciduria type III, which is characterized by early-onset bilateral optic atrophy, later-onset extrapyramidal dysfunction, and increased urinary excretion of 3-methylglutaconic acid and 3-methylglutaric acid [5].

In terms of its role in diseases, overexpression of OPA3 promotes colorectal cancer progression by inhibiting the cGAS-STING pathway mediated by mtDNA stress [3]. In ovarian cancer, high OPA3 expression is associated with poorer overall survival, and its inactivation is associated with increased sensitivity to certain drugs [2]. In doxorubicin-induced cardiotoxicity, hydrogen sulfide protects against it by regulating the OPA3-NFS1 axis, where OPA3 is involved in the regulation of ferroptosis by interacting with NFS1 [1]. In Plasmodium falciparum, the PfOPA3 protein is essential for maintaining mitochondrial homeostasis and parasite proliferation [4].

In conclusion, OPA3 is crucial for mitochondrial-related functions. Its study in various disease models, such as in cancer and cardiotoxicity, helps to understand the molecular mechanisms underlying these diseases. This knowledge may potentially lead to the development of new therapeutic strategies targeting OPA3 for treating related diseases.