Optic atrophy 1 mitochondrial dynamin-like GTPase (OPA1)

Optic atrophy 1 mitochondrial dynamin-like GTPase (OPA1) is an inner mitochondrial membrane–anchored, large dynamin superfamily GTPase that plays a central role in mitochondrial inner membrane fusion, preserving the structure and function of cristae, maintaining respiratory chain activity, and regulating mitochondrial network morphology. OPA1 is expressed as multiple tissue-specific isoforms due to alternative splicing and proteolytic processing, producing both long (membrane-anchored) and short (soluble) forms, both of which contribute to mitochondrial fusion and cristae maintenance. Mutations in OPA1 cause autosomal dominant optic atrophy, a hereditary neurodegenerative disease, and its altered function is implicated in mitochondrial disorders and heart failure. Therapeutic modulation is complex: direct targeting is not clinically established, but the protein’s abundance and proteolytic regulation are pursued as potential strategies for neuroprotection and metabolic disease.

Other names

OPA1OPA-1Optic atrophy type 1 proteinMitochondrial dynamin-like GTPaseDynamin-related protein OPA1

Enhancement or inhibition of OPA1 cleavage and splicing regulates mitochondrial fusion and cristae structure, indirectly affecting cell survival and energy production. Modulating activity of upstream mitochondrial proteases (OMA1, YME1L, PARL) that process OPA1