Rabbit anti-Human, Mouse OPA3 Polyclonal Antibody | anti-OPA3 antibody

OPA3 Antibody

Cat. Num. AAA7044387

• Gene Names: OPA3; MGA3
• Reactivity: Human, Mouse
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Antigen Affinity Purified
• Synonyms: OPA3; Polyclonal Antibody; OPA3 Antibody; Optic atrophy 3 protein; anti-OPA3 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Antigen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 180

• Applicable Applications for anti-OPA3 antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Species: Human
• Immunogen: Recombinant human Optic atrophy 3 protein (C-155AA)
• Conjugate: Non-conjugated
• 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.

Western Blot (WB)

(Western blotAll lanes: OPA3 antibody at 4.91 ug/ml+Mouse eye tissueSecondaryGoat polyclonal to rabbit IgG at 1/10000 dilutionPredicted band size: 20,21 kDaObserved band size: 20 kDa)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human kidney tissue using MBS7044387 at dilution 1:100)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human gastric cancer using MBS7044387 at dilution 1:100)

Related Product Information for anti-OPA3 antibody

May play some role in mitochondrial processes.

NCBI and Uniprot Product Information

• NCBI GI #: 156151427
• NCBI GeneID: 80207
• NCBI Accession #: NP_001017989.2
• NCBI GenBank Nucleotide #: NM_001017989.2
• UniProt Accession #: Q9H6K4; Q6P384; Q8N784
• 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
• NCBI Official Synonym Symbols: MGA3
• NCBI Protein Information: optic atrophy 3 protein
• UniProt Protein Name: Optic atrophy 3 protein
• Protein Family: OPA3-like protein
• UniProt Gene Name: OPA3
• UniProt Entry Name: OPA3_HUMAN

NCBI Description

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 Description

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