Anti-OPA3 Antibody (CAB7997)
- SKU:
- CAB7997
- Product type:
- Antibody
- Application:
- WB
- Application:
- IHC
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Signal Transduction
Frequently bought together:
Description
| 抗体名: | OPA3 Rabbit Polyclonal Antibody |
| 抗体コード: | CAB7997 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IHC |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 25-179 of human OPA3 (NP_079412.1). |
| 申し込み: | WB IHC |
| 推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | Mouse eye, Rat eye |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 25-179 of human OPA3 (NP_079412.1). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | 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 |
| 遺伝子ID: | 80207 |
| Uniprot: | Q9H6K4 |
| セルラーロケーション: | Mitochondrion |
| 計算された分子量: | 19kDa/20kDa |
| 観察された分子量: | 20kDa |
| 同義語: | OPA3, MGA3 |
| バックグラウンド: | 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. |
| 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 |
 | Western blot analysis of extracts of various cell lines, using OPA3 antibody (CAB7997) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
 | Immunohistochemistry of paraffin-embedded human stomach using OPA3 antibody (CAB7997) at dilution of 1:100 (40x lens). |
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