NUBPL antibody (AA 59-319)
Quick Overview for NUBPL antibody (AA 59-319) (ABIN7602094)
Target
See all NUBPL AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Binding Specificity
 - AA 59-319
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Purpose
 - Anti-NUBPL Antibody Picoband®
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Cross-Reactivity (Details)
 - No cross-reactivity with other proteins.
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Characteristics
 - Anti-NUBPL Antibody Picoband® (ABIN7602094). Tested in ELISA, IF, IHC, ICC, WB, Flow Cytometry applications. This antibody reacts with Human, Mouse. 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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Purification
 - Immunogen affinity purified.
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Immunogen
 - E.coli-derived human NUBPL recombinant protein (Position: K59-E319).
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Isotype
 - IgG
 
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Application Notes
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                        Western blot, 0.25-0.5 μg/mL, Human, Mouse
Immunohistochemistry, 2-5 μg/mL, Human
Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human
Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human
ELISA, 0.1-0.5 μg/mL, -
1. Calvo, S. E., Tucker, E. J., Compton, A. G., Kirby, D. M., Crawford, G., Burtt, N. P., Rivas, M., Guiducci, C., Bruno, D. L., Goldberger, O. A., Redman, M. C., Wiltshire, E., Wilson, C. J., Altshuler, D., Gabriel, S. B., Daly, M. J., Thorburn, D. R., Mootha, V. K. High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency. Nature Genet. 42: 851-858, 2010. 2. Dickinson, M. E., Flenniken, A. M., Ji, X., Teboul, L., Wong, M. D., White, J. K., Meehan, T. F., Weninger, W. J., Westerberg, H., Adissu, H., Baker, C. N., Bower, L., and 73 others. High-throughput discovery of novel developmental phenotypes. Nature 537: 508-514, 2016. Note: Erratum: Nature 551: 398 only, 2017. 3. Friederich, M. W., Perez, F. A., Knight, K. M., Van Hove, R. A., Yang, S. P., Saneto, R. P., Van Hove, J. L. K. Pathogenic variants in NUBPL result in failure to assemble the matrix arm of complex I and cause a complex leukoencephalopathy with thalamic involvement. Molec. Genet. Metab. 129: 236-242, 2020. - 
                                            
Restrictions
 - For Research Use only
 
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Format
 - Lyophilized
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Reconstitution
 - Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
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Concentration
 - 500 μg/mL
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Buffer
 - Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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Storage
 - 4 °C,-20 °C
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Storage Comment
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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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- NUBPL (Nucleotide Binding Protein-Like (NUBPL))
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Alternative Name
 - NUBPL
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Background
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Synonyms: RNA-binding protein Nova-2, Astrocytic NOVA1-like RNA-binding protein, Neuro-oncological ventral antigen 2, NOVA2, ANOVA, NOVA3
Tissue Specificity: Brain. Expression restricted to astrocytes.
Background: This gene encodes a member of the Mrp/NBP35 ATP-binding proteins family. The encoded protein is required for the assembly of the respiratory chain NADH dehydrogenase (complex I), an oligomeric enzymatic complex located in the inner mitochondrial membrane. Mutations in this gene cause mitochondrial complex I deficiency. Alternative splicing results in multiple transcript variants.
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Molecular Weight
 - 34 kDa
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Gene ID
 - 80224
 
Target
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