MyBioSource

protein

Ceruloplasmin protein

MBS537196

1 mg

330 USD

MBS537196

Native Protein

Ceruloplasmin protein

Ceruloplasmin

ceruloplasmin; Ceruloplasmin; ceruloplasmin; ceruloplasmin (ferroxidase); Ferroxidase

CP; CP; CP-2; N/A

CERU_HUMAN

Human plasma

Human

> 95% pure by SDS PAGE

Lyophilized from 50mM KH2PO4, pH 6.8, with 100mM KCl, 20mM E-amino caproic acid and 5mM EDTA. Reconstitute with deionized water to the pre-lyophilization volume.

Aliquot and store at -20 deg C. Avoid repeated freeze/thaw cycles

Biohazard Information: Donor samples were tested and found to be negative for HBsAg, anti-HCV, anti-HBc, and negative for anti-HIV 1 & 2. Nonetheless caution should be used when handling this material as there is a margin of error in all tests.

Nutrition & Metabolism; Proteins; Native & Recombinant Proteins; Native

Purified native Human Ceruloplasmin protein. An acute-phase reactant. Increased levels are associated with normal pregnancy, rheumatoid arthritis, and cirrhosis. Decreased levels are associated with hepatolenticular degeneration (Wilson's Disease). An elevated level of Cp is found in patients with progressive tumors. Additionally, as Cp is a prooxidant, an elevated level is a sign of cardiovascular disease.

1731685

BAA08085.1

P00450

132 kDa

HIF-1-alpha Transcription Factor Network Pathway 138045!!Iron Uptake And Transport Pathway 187191!!Metal Ion SLC Transporters Pathway 161066!!Porphyrin And Chlorophyll Metabolism Pathway 83021!!Porphyrin And Chlorophyll Metabolism Pathway 407!!SLC-mediated Transmembrane Transport Pathway 119558!!Transmembrane Transport Of Small Molecules Pathway 106572!!Transport Of Glucose And Other Sugars, Bile Salts And Organic Acids, Metal Ions And Amine Compounds Pathway 119571

The protein encoded by this gene is a metalloprotein that binds most of the copper in plasma and is involved in the peroxidation of Fe(II)transferrin to Fe(III) transferrin. Mutations in this gene cause aceruloplasminemia, which results in iron accumulation and tissue damage, and is associated with diabetes and neurologic abnormalities. Two transcript variants, one protein-coding and the other not protein-coding, have been found for this gene. [provided by RefSeq, Feb 2012]

Function: Ceruloplasmin is a blue, copper-binding (6-7 atoms per molecule) glycoprotein. It has ferroxidase activity oxidizing Fe2+ to Fe3+ without releasing radical oxygen species. It is involved in iron transport across the cell membrane. Provides Cu2+ 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 . By similarity. Catalytic activity: 4 Fe2+ + 4 H+ + O2 = 4 Fe3+ + 2 H2O. Cofactor: Binds 6 copper ions per monomer. Subcellular location: Secreted. Note: Colocalizes with GCP1 in secretory intracellular compartments . By similarity. Tissue specificity: Expressed by the liver and secreted in plasma. Involvement in disease: Defects in CP are the cause of aceruloplasminemia (ACERULOP) [. MIM:604290]. 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.Note=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. Sequence similarities: Belongs to the multicopper oxidase family.Contains 3 F5/8 type A domains.Contains 6 plastocyanin-like domains.

1 mg

330 USD