1998 Fiscal Year Final Research Report Summary
Defect of carbohydrate-deficient glycoprotein syndrome type I
Project/Area Number |
09680604
|
Research Category |
Grant-in-Aid for Scientific Research (C)
|
Allocation Type | Single-year Grants |
Section | 一般 |
Research Field |
Structural biochemistry
|
Research Institution | SASAKI INSTITUTE |
Principal Investigator |
OHKURA Takashi SASAKI INSTITUTE, DEPT.OF BIOCHEMISTRY, 生化学部, 主任研究員 (50183223)
|
Project Period (FY) |
1997 – 1998
|
Keywords | CDGS / lipid-linked oligosaccharide / mannose / GPI-anchor |
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.
|
Research Products
(14 results)
-
-
-
-
-
-
-
-
[Publications] Fukushima, K., Hara-Kuge, S., Ohkura, T., Seko, A.Ideo, H., Inazu, T., and Yamashita, K.: "Lectin-like characteristics of recombinant human interleukin- Ibeta recognizing glycans of the glycosylphosphatidylinositol anchor." J.Biol.Chem.272. 10579-10584 (1997)
Description
「研究成果報告書概要(欧文)」より
-
-
[Publications] Ohkura, T., Fukushima, K., Kurisaki, A., Sagami, H., Ogura, K., Ohno, K., Hara-Kuge, S., and Yamashita, K.: "A partial deficiency of dehydrodolichol reduction is a cause of carbohydrate-glycoprotein syndrome type I." J.Biol.Chem.272. 6868-6875 (1997)
Description
「研究成果報告書概要(欧文)」より
-
-
[Publications] Yoshikawa, K., Umetsu, K., Shinzawa, H., Yuasa, I., Ohkura, T., and Yamashita, K.: "Determination of carbohydrate-deficient transferrin separated by lectin affinity chromatography for detedting high alcohol consumption." Clin. Chem. (in press.).
Description
「研究成果報告書概要(欧文)」より
-
-