4ejx | pdb_00004ejx
From Proteopedia
Structure of ceruloplasmin-myeloperoxidase complex
| ||||||||||||
Structural highlights
DiseaseCERU_HUMAN 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. FunctionCERU_HUMAN 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 (By similarity). See Also | ||||||||||||||||||||
This page was last modified 15:03, 14 March 2024.