Factor I rabbit pAb

• 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 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 Ref

• Description:

  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 Ref

• Synonyms:

  CFI; IF; Complement factor I; C3B/C4B inactivator

• Gene ID:

  3426

• UniProt:

  P05156

• Cellular Locus:

  Secreted, extracellular space. Secreted.

• Host:

  Rabbit

• Species Reactivity:

  Human, Rat, Mouse,

• Reactivity:

  Human; Rat; Mouse

• Immunogen:

  The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490

• Clonality:

  Polyclonal

• Isotype:

  IgG

• Source:

  Rabbit

• Applications:

  WB, ELISA

• Validated Applications:

  WB, ELISA

• Stability:

  -20°C/1 year

• Concentration:

  1 mg/mL

• Dilution:

  IHC: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.

• Molecular Weight:

  Full lenth:66kD, heavy chain: 50-58kD

• Storage Conditions:

  PBS with 0.02% sodium azide and 50% glycerol pH 7.4. Store at -20°C. Avoid repeated freeze-thaw cycles.

• Observed Molecular Weight:

  Full - lenth:66 kD, heavy - chain: - 50 - 58 kD

• Subcellular Location:

  Secreted, extracellular space. Secreted .

• Other Product Names:

  CFI; IF; Complement factor I; C3B/C4B inactivator

• Gene ID (Human):

  3426

• SwissProt (Human):

  P05156