Sign in or Register   Sign in or Register
  |  

Mouse Anti-OPA3 Recombinant Antibody (CBXO-0473) (CBMAB-O0470-CQ)

This product is a mouse antibody that recognizes OPA3. The antibody CBXO-0473 can be used for immunoassay techniques such as: WB, IHC.
See all OPA3 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXO-0473
Antibody Isotype
IgG1
Application
WB, IHC

Basic Information

Immunogen
Full length human Recombinant protein of human OPA3(NP_079412) produced in E. coli
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.3, containing 1% BSA, 50% glycerol
Preservative
0.02% Sodium Azide
Concentration
10 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
OPA3, Outer Mitochondrial Membrane Lipid Metabolism Regulator
Introduction
The mouse ortholog of this protein co-purifies with the mitochondrial inner membrane. Mutations in this gene have been shown to result in 3-methylglutaconic aciduria type III and autosomal dominant optic atrophy and cataract. Multiple transcript variants encoding different isoforms have been found for this gene.
Entrez Gene ID
80207
UniProt ID
Q9H6K4
Alternative Names
OPA3, Outer Mitochondrial Membrane Lipid Metabolism Regulator; Optic Atrophy 3 (Autosomal Recessive, With Chorea And Spastic Paraplegia); Optic Atrophy 3 (Iraqi-Jewish Optic Atrophy Plus); Optic Atrophy 3 Protein; MGA3
Function
May play some role in mitochondrial processes.
Biological Process
Regulation of lipid metabolic processManual Assertion Based On ExperimentIBA:GO_Central
Response to stimulusIEA:UniProtKB-KW
Visual perceptionManual Assertion Based On ExperimentIMP:UniProtKB
Cellular Location
Mitochondrion
Involvement in disease
3-methylglutaconic aciduria 3 (MGCA3):
An autosomal recessive metabolic disorder that causes a neuro-ophthalmologic syndrome consisting of early-onset bilateral optic atrophy, spasticity, extrapyramidal dysfunction and cognitive deficit. Urinary excretion of 3-methylglutaconic acid and 3-methylglutaric acid is increased. MGCA3 can be distinguished from MGCA1 by the absence of increase of 3-hydroxyisovaleric acid levels.
Optic atrophy 3 (OPA3):
A condition that features progressive visual loss in association with optic atrophy. Atrophy of the optic disk indicates a deficiency in the number of nerve fibers which arise in the retina and converge to form the optic disk, optic nerve, optic chiasm and optic tracts. OPA3 is associated with cataract and a neurologic disorder characterized by extrapyramidal signs and ataxia.
Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-OPA3 Recombinant Antibody (CBXO-0473)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare