Anti-NFU1 Antibody Picoband®

About this product

Boster Bio Anti-NFU1 Antibody Picoband® catalog # A06191-1. Tested in WB, ICC/IF, Flow Cytometry, ELISA 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.

Specifications

Catalog number

A06191-1

Size / count

100 µg/Vial

Dry ice

No

Hazardous

No

Discontinued

No

Background

This gene encodes a protein that is localized to mitochondria and plays a critical role in iron-sulfur cluster biogenesis. The encoded protein assembles and transfers 4Fe-4S clusters to target apoproteins including succinate dehydrogenase and lipoic acid synthase. Mutations in this gene are a cause of multiple mitochondrial dysfunctions syndrome-1, and pseudogenes of this gene are located on the short arms of chromosomes 1 and 3. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene.

Synonyms

NFU1; HIRIP5; CGI-33; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial; HIRA-interacting protein 5

Gene Name

NFU1

Gene ID

27247

UniProt

Q9UMS0

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

E.coli-derived human NFU1 recombinant protein (Position: Q42-P254) . Human NFU1 shares 93% amino acid (aa) sequence identity with mouse NFU1.

Clonality

Polyclonal

Applications

WB, ICC/IF, Flow Cytometry, ELISA

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

References & Citations

1. Ahting, U., Mayr, J. A., Vanlander, A. V., Hardy, S. A., Santra, S., Makowski, C., Alston, C. L., Zimmermann, F. A., Abela, L., Plecko, B., Rohrbach, M., Spranger, S., and 12 others. Clinical, biochemical, and genetic spectrum of seven patients with NFU1 deficiency. Front. Genet. 6: 123, 2015. 2. Cameron, J. M., Janer, A., Levandovskiy, V., MacKay, N., Rouault, T. A., Tong, W.-H., Ogilvie, I., Shoubridge, E. A., Robinson, B. H. Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes. Am. J. Hum. Genet. 89: 486-495, 2011. 3. Ganesh, S., Tsurutani, N., Suzuki, T., Ueda, K., Agarwala, K. L., Osada, H., Delgado-Escueta, A. V., Yamakawa, K. The Lafora disease gene product laforin interacts with HIRIP5, a phylogenetically conserved protein containing a NifU-like domain. Hum. Molec. Genet. 12: 2359-2368, 2003.

Storage Conditions

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.

Calculated Molecular Weight

28 kDa

Observed Molecular Weight

28 kDa

Applications Notes

6

Gene Name Synonym

NFU1 iron-sulfur cluster scaffold

Protein Name

NFU1 iron-sulfur cluster scaffold homolog, mitochondrial

Isotype

Rabbit IgG

Contents

Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.