Product Name
OPA3, Polyclonal Antibody
Popular Item
Full Product Name
OPA3 Antibody
Product Synonym Names
Optic atrophy 3 protein; OPA3
Product Gene Name
anti-OPA3 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9H6K4
Species Reactivity
Human, Mouse
Purity/Purification
Antigen Affinity Purified
Immunogen
Recombinant human Optic atrophy 3 protein (C-155AA)
Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-82004 / sc-82006
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-OPA3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-OPA3 antibody
May play some role in mitochondrial processes.
Applications Tested/Suitable for anti-OPA3 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
Western Blot (WB) of anti-OPA3 antibody
Western blot
All lanes: OPA3 antibody at 4.91 ug/ml
+Mouse eye tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 20,21 kDa
Observed band size: 20 kDa
Immunohistochemistry (IHC) of anti-OPA3 antibody
Immunohistochemistry of paraffin-embedded human kidney tissue using MBS7044387 at dilution 1:100
Immunohistochemistry (IHC) of anti-OPA3 antibody
Immunohistochemistry of paraffin-embedded human gastric cancer using MBS7044387 at dilution 1:100
NCBI/Uniprot data below describe general gene information for OPA3. It may not necessarily be applicable to this product.
NCBI Accession #
NP_001017989.2
[Other Products]
NCBI GenBank Nucleotide #
NM_001017989.2
[Other Products]
UniProt Primary Accession #
Q9H6K4
[Other Products]
UniProt Secondary Accession #
Q6P384; Q8N784[Other Products]
UniProt Related Accession #
Q9H6K4[Other Products]
Molecular Weight
20,255 Da
NCBI Official Full Name
optic atrophy 3 protein isoform a
NCBI Official Synonym Full Names
optic atrophy 3 (autosomal recessive, with chorea and spastic paraplegia)
NCBI Official Symbol
OPA3 [Similar Products]
NCBI Official Synonym Symbols
MGA3
[Similar Products]
NCBI Protein Information
optic atrophy 3 protein
UniProt Protein Name
Optic atrophy 3 protein
Protein Family
OPA3-like protein
UniProt Gene Name
OPA3 [Similar Products]
UniProt Entry Name
OPA3_HUMAN
NCBI Summary for OPA3
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. [provided by RefSeq, Oct 2009]
UniProt Comments for OPA3
OPA3: May play some role in mitochondrial processes. Defects in OPA3 are the cause of 3-methylglutaconic aciduria type 3 (MGA3); also known as optic atrophy plus syndrome or Costeff optic atrophy syndrome. MGA3 is an autosomal recessive 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. MGA3 can be distinguished from MGA1 by the absence of increase of 3- hydroxyisovaleric acid levels. Defects in OPA3 are the cause of optic atrophy type 3 (OPA3); also known as autosomal dominant optic atrophy and cataract (ADOAC) or cataract, optic atrophy and neurologic disorder. Hereditary optic atrophy form a heterogeneous group of disorders. The autosomal dominant forms are characterized by an insidious onset of visual impairment in early childhood with moderate to severe loss of visual acuity, temporal optic disk pallor, color vision deficits and centrocecal scotoma of variable density. Belongs to the OPA3 family. 2 isoforms of the human protein are produced by alternative splicing.
Chromosomal Location of Human Ortholog: 19q13.32
Cellular Component: mitochondrion
Biological Process: regulation of lipid metabolic process; visual perception
Disease: 3-methylglutaconic Aciduria, Type Iii; Optic Atrophy 3, Autosomal Dominant
Research Articles on OPA3
1. Mutations of the OPA3 gene can cause either autosomal dominant or autosomal recessive optic atrophy.
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