Opa1 - OPA1, mitochondrial dynamin like GTPase

Predicted to enable several functions, including GTPase activity; anion binding activity; and microtubule binding activity. Involved in negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway and negative regulation of release of cytochrome c from mitochondria. Acts upstream of or within mitochondrial fusion and neural tube closure. Located in dendrite; mitochondrial inner membrane; and mitochondrial intermembrane space. Is expressed in several structures, including heart; liver; lung; skin; and visual system. Used to study optic atrophy. Human ortholog(s) of this gene implicated in Behr syndrome; dominant optic atrophy plus syndrome; mitochondrial DNA depletion syndrome 14; optic atrophy; and optic atrophy 1. Orthologous to human OPA1 (OPA1 mitochondrial dynamin like GTPase). [provided by Alliance of Genome Resources, Apr 2022]