|Budget Amount *help
¥2,900,000 (Direct Cost : ¥2,900,000)
Fiscal Year 1998 : ¥1,200,000 (Direct Cost : ¥1,200,000)
Fiscal Year 1997 : ¥1,700,000 (Direct Cost : ¥1,700,000)
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.