The genetic mutations causing Wilson disease impair the hepatic incorporation of copper into apoceruloplasmin, leading to a reduction in the total serum ceruloplasmin concentration due to the instability of the apoceruloplasmin relative to the holoprotein
with its full complement of copper [24,25].
Herms, "Involvement of presenilin holoprotein
upregulation in calcium dyshomeostasis of Alzheimer's disease," Journal of Cellular and Molecular Medicine, vol.
To ascertain sites where introduction of amino acid residues would be tolerated without affecting folding of the polypeptides and assembly into holoprotein
, methionine residues were introduced into different regions of the Gy1 gene (Utsumi et al.
13) in which apoproteins are first separated from holoproteins
and then the TC fraction of the holoproteins
is directly measured by ELISA.
They first separated holoproteins
from apoproteins by use of cobalamin-coated magnetic beads and then directly assayed the TC fraction of the holoproteins