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Anti-NUBPL Antibody Picoband® Fluoro647 Conjugated

Product Specifications

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.

Synonyms

RNA-binding protein Nova-2; Astrocytic NOVA1-like RNA-binding protein; Neuro-oncological ventral antigen 2; NOVA2; ANOVA; NOVA3

Gene Name

NUBPL

Gene ID

80224

UniProt

Q8TB37

Host

Rabbit

Reactivity

Human, Mouse

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E.coli-derived human NUBPL recombinant protein (Position: K59-E319) .

Clonality

Polyclonal

Tissue Specificity

Brain. Expression restricted to astrocytes.

Applications

Flow Cytometry

Field of Research

Channels, Metabolism, Plasma Membrane, Signal Transduction

Purification

Immunogen affinity purified.

Form

Liquid

Function

May regulate RNA splicing or metabolism in a specific subset of developing neurons. Binds single strand RNA.

References & Citations

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.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

29370 MW

Applications Notes

6

Gene Name Synonym

NOVA alternative splicing regulator 2

Subcellular Location

Nucleus.

Protein Name

Aquaporin-6

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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