| Research Abstract |
The most characteristic abnormality of fibroblasts derived from CDGS-I patients is the truncated lipid-linked oligosaccharides (LLO), that are easily affected by glycoproteins and free mannose in FCS.Since the size of LLO from CDGS-I cells is normalized by a addition of mannose, mannose concentrations required for the normalization of LLO were analyzed.As a result, the LLOs of healthy control cells were normalized at 50% in about 7 muM of mannose, however, 28, 48, and 42 muM for CDGS-I cells.It indicates that CDGS-I in Japan is caused by a defect of mannose pathway, probably phosphomannomutase.
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