Rabbit NFU1 Polyclonal Antibody | anti-NFU1 antibody

NFU1 Rabbit Polyclonal

Cat. Num. AAA7601947

• Gene Names: NFU1; Nfu; NifU; HIRIP; MMDS1; NIFUC; CGI-33; HIRIP5
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunofluorescence
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: NFU1; Polyclonal Antibody; NFU1 Rabbit Polyclonal; anti-NFU1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 254

• Applicable Applications for anti-NFU1 antibody: ELISA (EIA), Western Blot (WB), Immunofluorescence (IF)
• Immunogen: NFU1 iron-sulfur cluster scaffold homolog (S. cerevisiae)
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded mouse brain using MBS7601947 (NFU1 antibody) at dilution of 1:100)

Western Blot (WB)

(mouse heart tissue were subjected to SDS PAGE followed by western blot with MBS7601947 (NFU1 antibody) at dilution of 1:1000)

NCBI and Uniprot Product Information

• NCBI GI #: 50593021
• NCBI GeneID: 27247
• NCBI Accession #: NP_001002755.1
• NCBI GenBank Nucleotide #: NM_001002755.2
• Molecular Weight: 25,924 Da
• NCBI Official Full Name: NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform 2
• NCBI Official Synonym Full Names: NFU1 iron-sulfur cluster scaffold
• NCBI Official Symbol: NFU1
• NCBI Official Synonym Symbols: Nfu; NifU; HIRIP; MMDS1; NIFUC; CGI-33; HIRIP5
• NCBI Protein Information: NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
• 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

NCBI Description

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. [provided by RefSeq, Dec 2011]

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