High-resolution capillary zone electrophoresis for transferrin glycoform analysis associated with congenital disorders of glycosylation.

Publisher: Wiley-VCH Country of Publication: Germany NLM ID: 101088554 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1615-9314 (Electronic) Linking ISSN: 16159306 NLM ISO Abbreviation: J Sep Sci Subsets: MEDLINE

Original Publication: Weinheim, Germany : Wiley-VCH, c2001-

Congenital Disorders of Glycosylation/*blood ; Electrophoresis, Capillary/*methods ; Transferrin/*metabolism; Congenital Disorders of Glycosylation/diagnosis ; Glycosylation ; Humans ; Isoelectric Focusing ; Transferrin/chemistry

High-resolution capillary zone electrophoresis is used to assess the transferrin profile in serum of patients with eight different congenital disorders of glycosylation that represent type I, type II, and mixed type I/II disorders. Capillary zone electrophoresis data are compared to patterns obtained by gel isoelectric focusing. The high-resolution capillary zone electrophoresis method is shown to represent an effective tool to assess the diversity of transferrin patterns. Hypoglycosylated disialo-, monosialo-, and asialo-transferrin in type I cases can be distinguished from the corresponding underdesialylated transferrin glycoforms present in type II disorders. The latter can be separated from and detected ahead of their corresponding hypoglycosylated forms of type I patients. Both types of glycoforms are detected in sera of mixed type I/II patients. The assay has the potential to be used as screening method for congenital disorders of glycosylation. It can be run with a few microliters of serum when microvials are used.
(© 2018 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.)

Keywords: capillary electrophoresis; congenital disorder of glycosylation; desialylated transferrin; transferrin glycoforms

0 (Transferrin)

Date Created: 20180428 Date Completed: 20181018 Latest Revision: 20191210

20231215

10.1002/jssc.201800082

29701302