ETFDH Antibody
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中文名稱：ETFDH兔多克隆抗體
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貨號：CSB-PA619056ESR2HU
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規格：￥440
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圖片：
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Western blot
All lanes: ETFDH antibody at 3.56µg/ml + 293T whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 69, 63 kDa
Observed band size: 63 kDa -
Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA619056ESR2HU at dilution of 1:100 -
Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA619056ESR2HU at dilution of 1:100 -
Immunofluorescent analysis of PC-3 cells using CSB-PA619056ESR2HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) ETFDH Polyclonal antibody
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Uniprot No.：
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基因名：ETFDH
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別名：Electron transfer flavoprotein ubiquinone oxidoreductase antibody; Electron transfer flavoprotein-ubiquinone oxidoreductase antibody; electron transferring flavoprotein dehydrogenase antibody; Electron-transferring-flavoprotein dehydrogenase antibody; ETF dehydrogenase antibody; ETF QO antibody; ETF ubiquinone oxidoreductase antibody; ETF-QO antibody; ETF-ubiquinone oxidoreductase antibody; ETFD_HUMAN antibody; Etfdh antibody; MADD antibody; mitochondrial antibody
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial protein (318-617AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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產品提供形式：Liquid
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應用范圍：ELISA, WB, IHC, IF
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推薦稀釋比：
Application Recommended Dilution WB 1:500-1:2000 IHC 1:20-1:200 IF 1:50-1:200 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Accepts electrons from ETF and reduces ubiquinone.
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基因功能參考文獻：
- Neurite shortening and impairment in neurite growth was caused by a mutation in ETFDH. PMID: 27935074
- This study identified three novel compound heterozygous mutations of ETFDH gene in patients with late-onset multiple acyl-CoA dehydrogenase deficiency, and discussed the significant clinical heterogeneity among patients with similar genotype. PMID: 27000805
- ETFDH mutation is the causative gene in patients with adult-onset multiple acyl-CoA dehydrogenase deficiency with severe sensory neuropathy. PMID: 26821934
- Mtation c.250G>A and mutation c.353G>T in the ETFDH gene are associate with multiple aeyl-CoA dehydrogenase deficiency with severe fatty liver. PMID: 27060313
- identified 61 ETFDH mutations, including 31 novel mutations, which were widely distributed within the coding sequence PMID: 24357026
- Mutations in SLC22A5 and ETFDH are associated with riboflavin responsive-multiple acyl-CoA dehydrogenase deficiency. PMID: 25119904
- Results show that a predicted benign ETFDH missense variationc.158A>G in exon 2 causes exon skipping and degradation of ETFDH protein in patient samples. PMID: 24123825
- Case Report: ETF dehydrogenase mutations resulting in mild glutaric aciduria type II and complex II-III deficiency in liver and muscle. PMID: 21088898
- folding defects in the variant ETF-QO proteins and multiple acyl-CoA dehydrogenation deficiency PMID: 22611163
- a significant reduced expression of ETFDH was identified in the muscle of ETFDH-deficient patients; ETFDH deficiency is a major cause of riboflavin-responsive MADD in southern China, and c.250G>A is an important mutation PMID: 21347544
- High frequency of ETFDH c.250G>A mutation in Taiwanese patients with late-onset lipid storage myopathy PMID: 20370797
- 3 known (c.250G>A, c380T>A, c.524G>T) and 1 novel (c.1831G>A) ETFDH mutation were detected by high resolution melting analysis. The carrier frequency of the hotspot mutation, c.250G>A, in the Taiwanese population was found to be 1:125. PMID: 20138856
- lipid storage myopathy caused by ETFDH gene mutations. PMID: 19758981
- expression from a baculovirus vector and kinetic and spectral characteristics PMID: 12049629
- Mutations are identified by molecular analysis of 20 ETF:QO-deficient patients. Twenty-one different disease-causing mutations were identified on 36 of the 40 chromosomes. PMID: 12359134
- patients had autosomal recessive mutations in ETFDH, suggesting ETFDH deficiency leads to a secondary CoQ10 deficiency; results indicate that the late-onset form of glutaric aciduria type II & the myopathic form of CoQ10 deficiency are allelic diseases PMID: 17412732
- study identified ETFDH mutations in all members of a large series of patients with riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency PMID: 17584774
- We performed mutation analysis in four Taiwanese MADD patients. Three novel ETFDH mutations were identified in four patients and all harbored the p.A84T mutation PMID: 19249206
- Four novel mutations (3 missenses and 1 deletion) in ETFDH were found in Chinese families that presented with riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency. PMID: 19265687
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相關疾病：Glutaric aciduria 2C (GA2C)
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亞細胞定位：Mitochondrion inner membrane.
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蛋白家族：ETF-QO/FixC family
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數據庫鏈接：
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