Rabbit NFU1 Polyclonal Antibody | anti-NFU1 antibody

NFU1 Polyclonal Antibody

Cat. Num. AAA3011943

• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: NFU1; Polyclonal Antibody; NFU1 Polyclonal Antibody; NFU1 iron-sulfur cluster scaffold homolog; mitochondrial; HIRA-interacting protein 5; HIRIP5; CGI-33; anti-NFU1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: NFU1 polyclonal antibody detects endogenous levels of NFU1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 254

• Applicable Applications for anti-NFU1 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:2000; IHC: 1:50-1:200
• Immunogen: Recombinant full length Human NFU1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot analysis of extracts of various cell lines, using NFU1 antibody.)

Related Product Information for anti-NFU1 antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 50593021
• NCBI Accession #: NP_001002755.1
• NCBI GenBank Nucleotide #: NP_001002755.1
• UniProt Accession #: Q9UMS0
• Molecular Weight: ~ 28kDa
• NCBI Official Full Name: NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform 2
• UniProt Protein Name: NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
• Protein Family: NifU-like protein
• UniProt Gene Name: NFU1
• UniProt Synonym Gene Names: HIRIP5
• UniProt Entry Name: NFU1_HUMAN

Uniprot Description

NFU1: Iron-sulfur cluster scaffold protein which can assemble [4Fe-2S] clusters and deliver them to target proteins. Defects in NFU1 are the cause of multiple mitochondrial dysfunctions syndrome type 1 (MMDS1). A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes. Belongs to the NifU family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial

Chromosomal Location of Human Ortholog: 2p15-p13

Cellular Component: cytoplasm; cytosol; mitochondrion; nucleoplasm; nucleus

Molecular Function: 4 iron, 4 sulfur cluster binding; iron ion binding; protein binding

Biological Process: iron-sulfur cluster assembly

Disease: Multiple Mitochondrial Dysfunctions Syndrome 1