Human NFU1 Recombinant Protein (RPPB4091)

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SKU:
RPPB4091
Product type:
Recombinant Protein
Size:
20ug
Species:
Human
Target:
NFU1
Synonyms:
CGI-33
Synonyms:
HIRIP
Synonyms:
HIRIP5
Synonyms:
MMDS1
Source:
Escherichia Coli
Uniprot:
Q9UMS0
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Description

Product Name:Human NFU1 Recombinant Protein
Product Code:RPPB4091
Size:20µg
Species:Human
Target:NFU1
Synonyms:CGI-33, HIRIP, HIRIP5, MMDS1, Nfu, NifU, NIFUC, NFU1iron-sulfur cluster scaffold homolog, mitochondrial, HIRA-interacting protein 5.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered clear solution.
Formulation:NFU1 protein solution (0.5mg/ml) containing Phosphate buffer saline (pH 7.4), 20% glycerol and 1mM DTT.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSGAAAVAA GLRRRFCHML KNPYTIKKQP LHQFVQRPLF PLPAAFYHPVRYMFIQTQDT PNPNSLKFIP GKPVLETRTM DFPTPAAAFR SPLARQLFRI EGVKSVFFGP DFITVTKENEELDWNLLKPD IYATIMDFFA SGLPLVTEET PSGEAGSEED DEVVAMIKEL LDTRIRPTVQ EDGGDVIYKGFEDGIVQLKL QGSCTSCPSS IITLKNGIQN MLQFYIPEVE GVEQVMDDES DEKEANSP

NFU1 is a protein which is localized to mitochondria and plays a vital role in iron-sulfur cluster biogenesis. The NFU1 protein constructs and transfers 4Fe-4S clusters to target apoproteins including succinate dehydrogenase and lipoic acid synthase. NFU1 gene mutations cause multiple mitochondrial dysfunctions syndrome-1, and pseudogenes of the NFU1 gene are located on the short arms of chromosomes 1 and 3.

NFU1 Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 268 amino acids (10-254 a.a) and having a molecular mass of 29.9kDa.NFU1 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

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
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