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OPA1

optic atrophy 1 (autosomal dominant)

Dynamin-related GTPase required for mitochondrial fusion and regulation of apoptosis. May form a diffusion barrier for proteins stored in mitochondrial cristae. Proteolytic processing in response to intrinsic apoptotic signals may lead to disassembly of OPA1 oligomers and release of the caspase activator cytochrome C (CYCS) into the mitochondrial intermembrane space.Dynamin-like 120 kDa protein, form S1: Inactive form produced by cleavage at S1 position by OMA1 following stress conditions that induce loss of mitochondrial membrane potential, leading to negative regulation of mitochondrial fusion.

Gene Name: optic atrophy 1 (autosomal dominant)
Synonyms: OPA1, KIAA0567, MGM1, LargeG, NPG, NTG, Optic atrophy protein 1
Target Sequences: AB011139 BAA25493.1 O60313

Publications (6)

1
High-mobility group box 1 is essential for mitochondrial quality control. Tang D, Kang R, Livesey KM, Kroemer G, Billiar TR, Van Houten B, Zeh HJ, Lotze MT. Cell metabolism. 2011 13:701-11. (ICC, WB; Mouse) [PubMed:21641551] [PMC:PMC3293110]
2
Mitochondrial proteome remodelling in pressure overload-induced heart failure: the role of mitochondrial oxidative stress. Dai DF, Hsieh EJ, Liu Y, Chen T, Beyer RP, Chin MT, MacCoss MJ, Rabinovitch PS. Cardiovascular research. 2012 93:79-88. (WB; Mouse) [PubMed:22012956] [PMC:PMC3243039]
3
Thrombin-induced shedding of tumour endothelial marker 5 and exposure of its RGD motif are regulated by cell-surface protein disulfide-isomerase. Vallon M, Aubele P, Janssen KP, Essler M. The Biochemical journal. 2012 441:937-44. [PubMed:22013897]
4
Regulation of acid-base transporters by reactive oxygen species following mitochondrial fragmentation. Johnson D, Allman E, Nehrke K. American journal of physiology. Cell physiology. 2012 302:C1045-54. (WB; Rat) [PubMed:22237403] [PMC:PMC3330737]
5
Defects in mitochondrial dynamics and metabolomic signatures of evolving energetic stress in mouse models of familial Alzheimer's disease. Trushina E, Nemutlu E, Zhang S, Christensen T, Camp J, Mesa J, Siddiqui A, Tamura Y, Sesaki H, Wengenack TM, Dzeja PP, Poduslo JF. PloS one. 2012 7:e32737. (WB; Mouse) [PubMed:22393443] [PMC:PMC3290628]
6
ALS/FTD mutations in UBQLN2 are linked to mitochondrial dysfunction through loss-of-function in mitochondrial protein import. Brian C Lin, Trong H Phung, Nicole R Higgins, Jessie E Greenslade, Miguel A Prado, Daniel Finley, Mariusz Karbowski, Brian M Polster, Mervyn J Monteiro. Human molecular genetics. 2021 June;30:1230-1246. [Full Text Article] [PubMed:33891006] [PMC:PMC8212775] Related Antibodies: LS-C143234.
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OPA1 ELISA Kit
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0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
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OPA1 ELISA Kit
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Sandwich
96-Well Microplate
Rat
0.156 - 10 ng/ml
Colorimetric - 450nm (TMB)
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The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).