Signal Transduction Antibodies 2
Anti-OPA3 Antibody (CAB4995)
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- SKU:
- CAB4995
- Product Type:
- Antibody
- Antibody Type:
- Polyclonal Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- OPA3
- Synonyms:
- MGA3
Description
| Product Name: | OPA3 Rabbit pAb |
| Product Code: | CAB4995 |
| Size: | 20uL, 50uL, 100uL |
| Synonyms: | OPA3, MGA3 |
| Applications: | WB |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 25-179 of human OPA3 (NP_079412.1). |
| Applications: | WB |
| Recommended Dilutions: | WB 1:200 - 1:1000 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | HeLa, Mouse eye, Rat eye |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 25-179 of human OPA3 (NP_079412.1). |
| Purification Method: | Affinity purification |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | NRIK EAAR RSEF FKTY ICLP PAQL YHWV EMRT KMRI MGFR GTVI KPLN EEAA AELG AELL GEAT IFIV GGGC LVLE YWRH QAQQ RHKE EEQR AAWN ALRD EVGH LALA LEAL QAQV QAAP PQGA LEEL RTEL QEVR AQLC NPGR SASH AVPA SKK |
| Gene ID: | 80207 |
| Uniprot: | Q9H6K4 |
| Cellular Location: | Mitochondrion |
| Calculated MW: | 19kDa/20kDa |
| Observed MW: | 17kDa |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | 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 |
| NCBI Summary: | 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 Code: | Q9H6K4 |
| NCBI GenInfo Identifier: | 20139177 |
| NCBI Gene ID: | 80207 |
| NCBI Accession: | Q9H6K4.1 |
| UniProt Secondary Accession: | Q9H6K4,Q6P384, Q8N784, |
| UniProt Related Accession: | Q9H6K4 |
| Molecular Weight: | 179 |
| NCBI Full Name: | Optic atrophy 3 protein |
| NCBI 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 |
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