IGF system in children with congenital disorders of glycosylation

Summary Objective The function of IGF system components is affected by their glycosylation status in vitro. However, little is known about the role of glycosylation status of these components in vivo. In this study we determined the impact of glycosylation on the endocrine IGF system in children with the rare syndrome of congenital disorders of glycosylation (CDG). Design Analyses of serum samples from children with CDG and healthy controls. Patients Children with CDG (N = 12) were recruited as part of a separate clinical study of mannose therapy at the Mayo Clinic. Serum from control children (N = 11) were obtained as routine samples before discard. Measurements Levels and glycosylation state of components of the IGF system and ability to form physiologically relevant ternary complexes composed of IGF, IGFBP‐3, and an acid‐labile subunit (ALS). Results Serum levels of IGF‐1, IGF‐2, ALS, and IGFBP‐3 were reduced (P