Ceruloplasmin (CP)

Specification

• Cat#: THP-0276
• Product Name: Ceruloplasmin (CP)
• Description: Ceruloplasmin (CP) protein is produced by Rat Serum. Protein Concentration: Typically >0.5 mg/mL (lot specific). Rat Ceruloplasmin is an approximately 120 kDa monomeric serum protein that fuctions as a copper transporter. It is a positive acute phase reactant, the levels of which increase significantly in rats in response to inflammation, tissue injury and disease.
• Molecular Weight: 120 kDa
• Source: Rat Serum
• Introduction: Ceruloplasmin is a blue, copper-binding (6-7 atoms per molecule) glycoprotein. It has ferroxidase activity oxidizing Fe(2+) to Fe(3+) without releasing radical oxygen species. It is involved in iron transport across the cell membrane. Provides Cu(2+) ions for the ascorbate-mediated deaminase degradation of the heparan sulfate chains of GPC1. May also play a role in fetal lung development or pulmonary antioxidant defense. Defects in CP are the cause of aceruloplasminemia (ACERULOP). It is an autosomal recessive disorder of iron metabolism characterized by iron accumulation in the brain as well as visceral organs. Clinical features consist of the triad of retinal degeneration, diabetes mellitus and neurological disturbances. Ceruloplasmin levels are decreased in Wilson disease, in which copper cannot be incorporated into ceruloplasmin in liver because of defects in the copper-transporting ATPase 2. Belongs to the multicopper oxidase family.
• Application: It has ferroxidase activity oxidizing Fe(2+) to Fe(3+) without releasing radical oxygen species. It is involved in iron transport across the cell membrane. Provides Cu(2+) ions for the ascorbate-mediated deaminase degradation of the heparan sulfate chains of GPC1. May also play a role in fetal lung development or pulmonary antioxidant defense.