OPA3 Antibody

說明書

中文名稱：

OPA3兔多克隆抗體
貨號：

CSB-PA880985ESR1HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA880985ESR1HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human gastric cancer using CSB-PA880985ESR1HU at dilution of 1:100

Immunofluorescent analysis of Hela cells using CSB-PA880985ESR1HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) OPA3 Polyclonal antibody
Uniprot No.：

Q9H6K4
基因名：

OPA3
別名：

OPA3 antibody; Optic atrophy 3 protein antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Optic atrophy 3 protein (25-179AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC, IF

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200
IF	1:50-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

May play some role in mitochondrial processes.
基因功能參考文獻：
1. Mutations of the OPA3 gene can cause either autosomal dominant or autosomal recessive optic atrophy. PMID: 25205859
2. Report the results of a comprehensive study on OPA3 mutations, including the mutation spectrum and its prevalence in a large cohort of ADOA patients, the associated clinical phenotype and the functional characterisation of a newly identified OPA3 mutant. PMID: 24136862
3. A novel missense mutation identifies OPA3 as the cause of a complex neurological disorder with marked lower limb dystonia. PMID: 23700088
4. OPA1 mutations are the most common genetic defects identified in patients with suspected Autosomal-dominant optic atrophy (DOA), whereas OPA3 mutations are very rare in isolated optic atrophy cases. PMID: 21036400
5. OPA3, as an integral mitochondrial outer membane perotein, has a crucial role in mitochondrial fission, and provides a direct link between mitochondrial morphology and optic atrophy. PMID: 20372962
6. findings thus place the cellular metabolic defect of 3-methylglutaconic aciduria type III in the mitochondrion rather than the peroxisome and implicate loss of OPA3A rather than gain of OPA3B in disease etiology. PMID: 20350831
7. two missense mutations in OPA3 in two families affected by autosomal dominant optic atrophy and cataract (ADOAC) PMID: 15342707
8. patient with Costeff syndrome presenting the characteristics of this syndrome and the single-nucleotide polymorphic gene that causes this disease. PMID: 16838891
9. OPA1 mutations, mtDNA mutations, and OPA3 mutations in 980 patients Leber's hereditary optic neuropathy and autosomal dominant optic atrophy PMID: 19319978
10. The mouse ortholog of OPA3 purifies with mitochondrial inner membranes. PMID: 12865426
11. The mouse ortholog of OPA3 purifies with mitochondria PMID: 18614015
12. type III 3-methylglutaconic aciduria (optic atrophy plus syndrome, or Costeff optic atrophy syndrome): identification of the OPA3 gene and its founder mutation in Iraqi Jews PMID: 11668429
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相關疾病：

3-methylglutaconic aciduria 3 (MGA3); Optic atrophy 3 (OPA3)
亞細胞定位：

Mitochondrion.
蛋白家族：

OPA3 family
組織特異性：

Ubiquitous. Most prominent expression in skeletal muscle and kidney.
數據庫鏈接：

HGNC: 8142

OMIM: 165300

KEGG: hsa:80207

UniGene: Hs.466945

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