Factor I (CFI) (NM_000204) Human Mass Spec Standard

CAT#: PH316645

CFI MS Standard C13 and N15-labeled recombinant protein (NP_000195)



  View other "Factor I" proteins (3)

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CNY 19,520.00


货期*
4周

规格
    • 10 ug

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Specifications

Product Data
Description CFI MS Standard C13 and N15-labeled recombinant protein (NP_000195)
Species Human
Expression Host HEK293
Expression cDNA Clone or AA Sequence RC216645
Predicted MW 65.72 kDa
Protein Sequence
Tag C-Myc/DDK
Purity > 80% as determined by SDS-PAGE and Coomassie blue staining
Concentration >0.05 µg/µL as determined by microplate BCA method
Labeling Method Labeled with [U- 13C6, 15N4]-L-Arginine and [U- 13C6, 15N2]-L-Lysine
Buffer 25 mM Tris-HCl, 100 mM glycine, pH 7.3
Reference Data
RefSeq NP_000195
RefSeq Size 1963
RefSeq ORF 1749
Synonyms AHUS3; ARMD13; C3b-INA; C3BINA; FI; IF; KAF
Locus ID 3426
Cytogenetics 4q25
Summary This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by RefSeq, Dec 2015]
Protein Families Druggable Genome, Protease, Secreted Protein
Protein Pathways Complement and coagulation cascades
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