OXA1L Anticorpi

OXA1L (oxidase (cytochrome c) assembly 1-like), also known as OXA1, is a 435 mitochondrial inner membrane protein belonging to the evolutionarily conserved Oxa1/Alb3/YidC protein family. Members of the Oxa1/Alb3/YidC protein family are involved in the biogenesis of membrane proteins in mitochondria, chloroplasts and bacteria. Existing as three isoforms produced by alternative splicing events, OXA1L is required for the insertion of integral membrane proteins into the mitochondrial inner membrane. OXA1L is essential for the activity and assembly of cytochrome oxidase and for the correct biogenesis of F(1)F(o)-ATP synthase and NADH:ubiquinone oxidoreductase. Mutations in the gene encoding OXA1L might be involved in the pathology of combined enzymatic deficiencies of the oxidative phosphorylation (OXPHOS) system.