OPA1 (D6U6N) Rabbit mAb

Changes in mitochondrial dynamics regulated by environmental cues affect mitochondrial size and shape and have been shown to dramatically impact mitochondrial metabolism, apoptosis, and autophagy (1). These processes are largely controlled by mitochondrial dynamin-related GTPases, including mitofusin-1, mitofusin-2, OPA1, and DRP1. DRP1 regulates mitochondrial fission, while the mitofusins and OPA1 control fusion at the outer and inner mitochondrial membrane, respectively.OPA1, or Optic Atrophy 1, was originally identified as a genetic cause for Autosomal Dominant Optic Atrophy, a neuropathy resulting in progressive visual loss (2,3). OPA1 is a widely expressed protein localized to the inner mitochondrial membrane, which regulates mitochondrial fusion and cristae morphology and protects against apoptosis (4-6). OPA1 activity is tightly regulated through alternative splicing and post-translational modifications including complex proteolytic processing by multiple proteases (7-12). In addition, OPA1 expression can be induced under conditions of metabolic demand through a pathway involving Parkin induced NF-κB activation (13). 1.Kasahara, A. and Scorrano, L. (2014) Trends Cell Biol 24, 761-70. 2.Delettre, C. et al. (2000) Nat Genet 26, 207-10. 3.Alexander, C. et al. (2000) Nat Genet 26, 211-5. 4.Frezza, C. et al. (2006) Cell 126, 177-89. 5.Olichon, A. et al. (2003) J Biol Chem 278, 7743-6. 6.Griparic, L. et al. (2004) J Biol Chem 279, 18792-8. 7.Delettre, C. et al. (2001) Hum Genet 109, 584-91. 8.Olichon, A. et al. (2007) Cell Death Differ 14, 682-92. 9.Ishihara, N. et al. (2006) EMBO J 25, 2966-77. 10.Cipolat, S. et al. (2006) Cell 126, 163-75. 11.Griparic, L. et al. (2007) J Cell Biol 178, 757-64. 12.Merkwirth, C. et al. (2008) Genes Dev 22, 476-88. 13.Müller-Rischart, A.K. et al. (2013) Mol Cell 49, 908-21.