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

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.

Evaluation of a commercially available carbohydrate deficient transferrin kit to detect beta-2-transferrin in cerebrospinal fluid using capillary electrophoresis.

OBJECTIVE: The aim of this study was to evaluate the CEofix™ CDT Capillary Electrophoresis (CE) kit for the detection of ß-2-tranferrin (ß-2-Tf) in cerebrospinal fluid (CSF). DESIGN AND METHOD: Evaluation was performed according to CLSI EP5-A and EP12-A guidelines. RESULTS: The method resolved ß-2-Tf from other Tf isoforms. The C50 for ß-Tf was determined to be 0.46 mg/L. Neither hemoglobin nor bilirubin co-migrated with ß-2-Tf. CONCLUSIONS: The CDT kit can be used for detecting ß-2-Tf in CSF by CE.