IF antibody (clone 1B3), anti-human
€435.00
In stock
SKU
AC-AT2482a
Catalog Number: AC-AT2482a
Size: 100 µg
Isotype: mouse IgG2b Kappa
Applications: WB, E
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Size: 100 µg
Isotype: mouse IgG2b Kappa
Applications: WB, E
Request Information AC-AT2482a">Request Information
Background:
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 uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene.
Other Names:
Complement factor I, C3B/C4B inactivator, Complement factor I heavy chain, Complement factor I light chain, CFI, IF
Antigen Type:
Recombinant Protein
Gene Name: CFI
Gene ID: 3426
NCBI Accession: NP_000195.2
Primary Accession: P05156
Other Accession: NM_000204
Format: Clear, colorless solution in phosphate buffered saline, pH 7.2 .
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 uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene.
Other Names:
Complement factor I, C3B/C4B inactivator, Complement factor I heavy chain, Complement factor I light chain, CFI, IF
Antigen Type:
Recombinant Protein
Gene Name: CFI
Gene ID: 3426
NCBI Accession: NP_000195.2
Primary Accession: P05156
Other Accession: NM_000204
Format: Clear, colorless solution in phosphate buffered saline, pH 7.2 .
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