Oral ingestion of mannose elevates blood mannose levels: a first step toward a potential therapy for carbohydrate-deficient glycoprotein syndrome type I
G Alton, S Kjaergaard, JR Etchison, F Skovby… - … and molecular medicine, 1997 - Elsevier
Carbohydrate-deficient glycoprotein syndrome type I (CDGS) is an inherited metabolic
disorder with multisystemic abnormalities resulting from a failure to add entire N-linked
oligosaccharide chains to many glycoproteins. Fibroblasts from these patients also
abnormally glycosylate proteins, but this lesion is corrected by providing 250 μm mannose to
the culture medium. This correction of protein glycosylation suggests that providing dietary
mannose to elevate blood mannose concentrations might also remedy some of the …
disorder with multisystemic abnormalities resulting from a failure to add entire N-linked
oligosaccharide chains to many glycoproteins. Fibroblasts from these patients also
abnormally glycosylate proteins, but this lesion is corrected by providing 250 μm mannose to
the culture medium. This correction of protein glycosylation suggests that providing dietary
mannose to elevate blood mannose concentrations might also remedy some of the …