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.
Categories
Primary Antibodies
Clonality
polyclonal
Description
Iron-sulfur cluster scaffold protein which can assemble [4Fe-2S] clusters and deliver them to target proteins.
Host
Rabbit
Immunogen
NFU1 iron-sulfur cluster scaffold homolog
Isotype
IgG
Molecular Weight
25 kDa
Reactivity
Human, Mouse, Rat
Regulatory
RUO
Synonyms
HIRIP5
Uniprot
Q9UMS0
Gene Id
27247
Research Area
Epigenetics
Form
liquid
Format
liquid
Purification
Immunogen affinity purified
Purity
>=95% as determined by SDS-PAGE
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months (Avoid repeated freeze / thaw cycles.)