Rabbit OPA3 Polyclonal Antibody | anti-OPA3 antibody

OPA3 Polyclonal Antibody

Cat. Num. AAA9433761

• Gene Names: OPA3; MGA3
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: Affinity purification
• Synonyms: OPA3; Polyclonal Antibody; OPA3 Polyclonal Antibody; MGA3; optic atrophy 3 protein; anti-OPA3 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Affinity purification
• Form/Format: PBS with 50% glycerol, pH7.4

• Applicable Applications for anti-OPA3 antibody: Western Blot (WB)
• Application Notes: WB: 1:200-1:1000
• Immunogen: Recombinant fusion protein of human OPA3 (NP_079412.1).
• Conjugation: Unconjugated
• Preparation and Storage: Store at -20 degree C. Avoid freeze/thaw cycles.

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using OPA3 antibody.)

Related Product Information for anti-OPA3 antibody

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.

Product Categories/Family for anti-OPA3 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 13376717
• NCBI GeneID: 80207
• NCBI Accession #: NP_079412.1
• NCBI GenBank Nucleotide #: NM_025136.3
• UniProt Accession #: Q9H6K4; Q6P384; Q8N784
• Molecular Weight: 20,255 Da
• NCBI Official Full Name: optic atrophy 3 protein isoform b
• 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; Optic atrophy 3 (Iraqi-Jewish 'optic atrophy plus')
• 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; neuromuscular process; response to stimulus; growth

Disease: Optic Atrophy 3, Autosomal Dominant; 3-methylglutaconic Aciduria, Type Iii