Anti-NFU1 Antibody Picoband®

SKU: A06191-1

Size: 100 μg

Storage: 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.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,ICC/IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml

Gene Name: NFU1

Specificity:

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.

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

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

Purification: Immunogen affinity purified.

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

Reference: 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.

Uniprot ID: Q9UMS0

Host: Rabbit

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

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 28 kDa

Calculated Molecular Weight: 28 kDa

Gene ID: 27247

Protein Name: NFU1 iron-sulfur cluster scaffold homolog, mitochondrial

Gene Full Name: NFU1 iron-sulfur cluster scaffold

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