Antibody Name:	Anti-NFU1 Antibody
Antibody SKU:	CAB7097
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-230 of human NFU1 (NP_001002755.1).

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	BT-474, SW480, THP-1, U-937, Mouse liver, Mouse heart, Mouse skeletal muscle, Rat liver, Rat heart

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-230 of human NFU1 (NP_001002755.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	MAAT ARRG WGAA AVAA GLRR RFCH MLKN PYTI KKQP LHQF VQRP LFPL PAAF YHPV RYMF IQTQ DTPN PNSL KFIP GKPV LETR TMDF PTPA AAFR SPLA RQLF RIEG VKSV FFGP DFIT VTKE NEEL DWNL LKPD IYAT IMDF FASG LPLV TEET PSGE AGSE EDDE VVAM IKEL LDTR IRPT VQED GGDV IYKG FEDG IVQL KLQG SCTS CPSS IITL KNGI QNML QF
Gene ID:	27247
Uniprot:	Q9UMS0
Cellular Location:	Cytoplasm, Mitochondrion, cytosol
Calculated MW:	12kDa/25kDa/28kDa
Observed MW:	25-28kDa

Synonyms:

NFU1, CGI-33, HIRIP, HIRIP5, MMDS1, NIFUC, Nfu, NifU

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.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 2p15-p13 Cellular Component: nucleoplasm; mitochondrion; cytoplasm; cytosol; nucleus Molecular Function:protein binding; 4 iron, 4 sulfur cluster binding; iron ion binding Biological Process: iron-sulfur cluster assembly Disease: Multiple Mitochondrial Dysfunctions Syndrome 1
NCBI Summary:	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 Code:	Q9UMS0
NCBI GenInfo Identifier:	205371805
NCBI Gene ID:	27247
NCBI Accession:	Q9UMS0.2
UniProt Secondary Accession:	Q9UMS0,Q53QE5, Q6VNZ8, Q7Z5B1, Q7Z5B2, Q9Y322, B4DUL9
UniProt Related Accession:	Q9UMS0
Molecular Weight:	254
NCBI Full Name:	NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
NCBI 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; HIRA-interacting protein 5; iron-sulfur cluster scaffold protein; NifU-like C-terminal domain containing
UniProt Protein Name:	NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
UniProt Synonym Protein Names:	HIRA-interacting protein 5
Protein Family:	NifU-like protein
UniProt Gene Name:	NFU1
UniProt Entry Name:	NFU1_HUMAN

	Western blot analysis of extracts of various cell lines, using NFU1 antibody (CAB7097) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
	Immunohistochemistry of paraffin-embedded mouse intestine using NFU1 Antibody (CAB7097) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse brain using NFU1 Antibody (CAB7097) at dilution of 1:100 (40x lens).