NDUFV2 抗体 (AA 1-249)
Quick Overview for NDUFV2 抗体 (AA 1-249) (ABIN7599154)
抗原
See all NDUFV2 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
 - AA 1-249
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原理
 - Anti-NDUFV2 Antibody Picoband®
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交叉反应 (详细)
 - No cross-reactivity with other proteins.
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产品特性
 - Anti-NDUFV2 Antibody Picoband® (ABIN7599154). Tested in ELISA, Flow Cytometry, IF, IHC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
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纯化方法
 - Immunogen affinity purified.
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免疫原
 - E.coli-derived human NDUFV2 recombinant protein (Position: M1-L249).
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亚型
 - IgG
 
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应用备注
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                        Western blot, 0.1-0.25 μg/mL/mL, Human, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/mL/mL, Human
Immunofluorescence, 5 μg/mL, Human
Flow Cytometry (Fixed), 1-3 μg/mL/1x10^6 cells, Human
ELISA, 0.1-0.5 μg/mL/mL, Human
1. Benit, P., Beugnot, R., Chretien, D., Giurgea, I., De Lonlay-Debeney, P., Issartel, J.-P., Corral-Debrinski, M., Kerscher, S., Rustin, P., Rotig, A., Munnich, A. Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy. Hum. Mutat. 21: 582-586, 2003. 2. Cameron, J. M., MacKay, N., Feigenbaum, A., Tarnopolsky, M., Blaser, S., Robinson, B. H., Schulze, A. Exome sequencing identifies complex I NDUFV2 mutations as a novel cause of Leigh syndrome. Europ. J. Paediat. Neurol. 19: 525-532, 2015. 3. de Coo, R., Buddiger, P., Smeets, H., Geurts van Kessel, A., Morgan-Hughes, J., Weghuis, D. O., Overhauser, J., van Oost, B. Molecular cloning and characterization of the active human mitochondrial NADH:ubiquinone oxidoreductase 24- kDa gene (NDUFV2) and its pseudogene. Genomics 26: 461-466, 1995. - 
                                            
限制
 - 仅限研究用
 
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状态
 - Lyophilized
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溶解方式
 - Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
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浓度
 - 500 μg/mL
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缓冲液
 - Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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储存条件
 - 4 °C,-20 °C
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储存方法
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                        At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. 
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing. 
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- NDUFV2 (NADH Dehydrogenase (Ubiquinone) Flavoprotein 2, 24kDa (NDUFV2))
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别名
 - NDUFV2
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背景
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Synonyms: Ubiquitin carboxyl-terminal hydrolase 16, Deubiquitinating enzyme 16, Ubiquitin thioesterase 16, Ubiquitin-processing protease UBP-M, Ubiquitin-specific-processing protease 16, USP16, MSTP039
Tissue Specificity: Present in all the tissues examined including fetal brain, lung, liver, kidney, and adult heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
Background: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (NDUFV2) is an enzyme that in humans is encoded by the NDUFV2 gene. The NADH-ubiquinone oxidoreductase complex (complex I) of the mitochondrial respiratory chain catalyzes the transfer of electrons from NADH to ubiquinone, and consists of at least 43 subunits. The complex is located in the inner mitochondrial membrane. This gene encodes the 24 kDa subunit of complex I, and is involved in electron transfer. Mutations in this gene are implicated in Parkinson's disease, bipolar disorder, schizophrenia, and have been found in one case of early onset hypertrophic cardiomyopathy and encephalopathy. A non-transcribed pseudogene of this locus is found on chromosome 19.
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分子量
 - 27 kDa
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基因ID
 - 4729
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UniProt
 - P19404
 
抗原
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