Code
OSO00042W
$240USD
ID Tag
Rb1363-140310-WS
Immunogen
A synthetic peptide from n-terminal region of human OPA1 conjugated to blue carrier protein was used as the antigen.
Accession
Also known
Dynamin-like 120 kDa protein, mitochondrial, Optic atrophy protein 1, Dynamin-like 120 kDa protein, form S1
Target
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.
Defects in OPA1 are a cause of optic atrophy type 1 (OPA1). OPA1 is a dominantly inherited optic neuropathy occurring in 1 in 50,000 individuals that features progressive loss in visual acuity leading, in many cases, to legal blindness. Defects in OPA1 are the cause of optic atrophy 1 with deafness. Some individuals with mutations in OPA1 manifest also ophthalmoplegia and myopathy.
SUBCELLULAR LOCATION: Mitochondrion inner membrane; Single-pass membrane protein. Mitochondrion intermembrane space
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.
Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Nagase T, et al. DNA Res. 5:31-39(1998)
Limitation
For research use only
Code
OSO00042W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for OPA1.
Spcs X-react.
Human, rat, mouse. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.