- Catalog number
- PB9731
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- Dynamin-like 120 kDa protein, mitochondrial is a protein that in humans is encoded by the OPA1 gene. It is mapped to 3q29. This protein regulates mitochondrial fusion and cristae structure in the inner mitochondrial membrane (IMM) and contributes to ATP synthesis and apoptosis. This gene product is a nuclear-encoded mitochondrial protein with similarity to dynamin-related GTPases. It is a component of the mitochondrial network. Mutations in this gene have been associated with optic atrophy type 1, which is a dominantly inherited optic neuropathy resulting in progressive loss of visual acuity, leading in many cases to legal blindness. Multiple transcript variants encoding different isoforms have been found for this gene.
- Synonyms
- Dynamin-like 120 kDa protein, mitochondrial;3.6.5.5; Optic atrophy protein 1; Dynamin-like 120 kDa protein, form S1; OPA1; KIAA0567
- Gene Name
- OPA1
- Gene ID
- 4976
- UniProt
- O60313
- Host
- Rabbit
- Reactivity
- Human, Mouse, Rat
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- A synthetic peptide corresponding to a sequence at the C-terminus of human OPA1, different from the related mouse and rat sequences by one amino acid.
- Clonality
- Polyclonal
- Tissue Specificity
- Highly expressed in retina. Also expressed in brain, testis, heart and skeletal muscle. Isoform 1 expressed in retina, skeletal muscle, heart, lung, ovary, colon, thyroid gland, leukocytes and fetal brain. Isoform 2 expressed in colon, liver, kidney, thyroid gland and leukocytes. Low levels of all isoforms expressed in a variety of tissues. .
- Applications
- Flow Cytometry, IF, IHC, IHC-F, ICC, WB
- Field of Research
- Cardiovascular, Metabolism, Mitochondrial Metabolism, Neurodegenerative Disease, Neurology Process, Neuroscience, Pathways and Processes, Sensory System, Visual System
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- 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. May also play a role in mitochondrial genome maintenance. .
- References & Citations
- 1. Patten DA, Wong J, Khacho M, Soubannier V, Mailloux RJ, Pilon-Larose K, MacLaurin JG, Park DS, McBride HM, Trinkle-Mulcahy L, Harper ME, Germain M, Slack RS (Nov 2014) . OPA1-dependent cristae modulation is essential for cellular adaptation to metabolic demand. The EMBO Journal 33 (22) : 2676–91. 2. Santarelli R, Rossi R, Scimemi P, Cama E, Valentino ML, La Morgia C, Caporali L, Liguori R, Magnavita V, Monteleone A, Biscaro A, Arslan E, Carelli V (Mar 2015) . OPA1-related auditory neuropathy: site of lesion and outcome of cochlear implantation. Brain 138 (Pt 3) : 563–76.
- Storage Conditions
- Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 111631 MW
- Observed Molecular Weight
- 80-100 kDa
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- 6
- Gene Name Synonym
- Dynamin-like 120 kDa protein, mitochondrial
- Subcellular Location
- Mitochondrion inner membrane ; Single-pass membrane protein . Mitochondrion intermembrane space .
- Protein Name
- Dynamin-like 120 kDa protein, mitochondrial
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.