ATP7B directs copper to plasma ceruloplasmin or to biliary excretion in concert with a newly discovered chaperone, Murr1, the protein missing in canine copper toxicosis.
22.
The disease involves poor incorporation of copper into ceruloplasmin and impaired biliary copper excretion and is usually induced by mutations impairing the function of the Wilson copper ATPase.
23.
Copper accumulates in the liver tissue; ceruloplasmin is still secreted, but in a form that lacks copper ( termed apoceruloplasmin ) and is rapidly degraded in the bloodstream.
24.
Substances that may impart a greenish hue to one's skin include biliverdin, the green pigment in bile, and ceruloplasmin, a protein that carries copper ions in chelation.
25.
Mutations in the ceruloplasmin gene ( " CP " ), which are very rare, can lead to the genetic disease aceruloplasminemia, characterized by hyperferritinemia with iron overload.
26.
Ceruloplasmin is also an acute-phase protein produced by monocytes, and its plasma concentration can double during multiple inflammatory conditions through increased Cp production by monocytic cells after stimulation by IFN-gamma.
27.
Copper is an integral part of the antioxidant enzyme, copper-zinc superoxide dismutase ( Cu, Zn-SOD ), and has a role in iron homeostasis as a cofactor in ceruloplasmin.
28.
Harbour developed a keen interest in research during his undergraduate years at Texas A & M, where he studied the function of the copper protein ceruloplasmin in the laboratory of Dr . Edward Harris.
29.
Apoceruloplasmin is largely degraded intracellularly in the hepatocyte and the small amount that is released has a short circulation half life of 5 hours as compared to the 5.5 days for the holo-ceruloplasmin.
30.
They demonstrated that recombinant human hephaestin ( rhHp ) bound copper ( determined by inductively coupled plasma mass spectrometry ) and exhibited an absorption maximum at ~ 610 nm consistent with other blue multicopper oxidases such as ceruloplasmin.
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