Anti-NFU1 Antibody Picoband® HRP Conjugated
Product Specifications
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, IHC, ELISA
Purification
Immunogen affinity purified.
Form
Liquid
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. Avoid repeated freezing and thawing.
Calculated Molecular Weight
28 kDa
Gene Name Synonym
NFU1 iron-sulfur cluster scaffold
Protein Name
NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4.
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