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Cubilin P1297L mutation associated with hereditary
megaloblastic anemia 1 causes impaired recognition of
intrinsic factor–vitamin B12 by cubilin
by Mette Kristiansen, Maria Aminoff, Christian Jacobsen, Albert de la Chapelle, Ralf
Krahe, Pierre J. Verroust, and Søren K. Moestrup
Blood
Volume 96(2):405-409
July 15, 2000
©2000 by American Society of Hematology
Mammalian expression of the IF–Cbl-binding region of wild-type and FM1 mutant cubilin.(A) The
modular protein organization of human cubilin (460 kd) and the IF–Cbl-binding region (cubilin
CUB domains 5-8, amino acid 928-1386) expressed in CHO cells stably t...
Mette Kristiansen et al. Blood 2000;96:405-409
©2000 by American Society of Hematology
Elution profiles of IF–Cbl-purified wild-type and FM1 mutant CUB 5-8 proteins.Nonreducing
SDS–PAGE (silver staining) of the eluted fractions.
Mette Kristiansen et al. Blood 2000;96:405-409
©2000 by American Society of Hematology
Proteolytic digestion of the wild-type and FM1 mutant CUB 5-8 proteins.Analysis by reducing
SDS–PAGE and silver staining.
Mette Kristiansen et al. Blood 2000;96:405-409
©2000 by American Society of Hematology
Surface plasmon resonance analysis of the binding of IF–Cbl flowing along BIAcore CM5 sensor
chips with immobilized wild-type or FM1 mutant form of CUB 5-8.(A) The IF-Cbl binding to the
wild-type CUB 5-8 protein with a flow concentration of 10, 20, and 50 n...
Mette Kristiansen et al. Blood 2000;96:405-409
©2000 by American Society of Hematology
Uptake of 125I–IF–Cbl in cubilin-expressing epithelial cells and inhibition with the wild-type and
FM1 mutant CUB 5-8 proteins.(A) Time-course for cell association (•) and degradation (○) of
iodine 125–IF–Cbl.
Mette Kristiansen et al. Blood 2000;96:405-409
©2000 by American Society of Hematology
Effect of wild-type and FM1 mutant CUB 5-8 proteins on the total uptake of 125I–IF–Cbl in
epithelial cells.Values are the mean ± 1 SD of triplicate experiments and are shown as the
percentage of uptake in relation to the control uptake of iodine 125–IF–Cbl ...
Mette Kristiansen et al. Blood 2000;96:405-409
©2000 by American Society of Hematology
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