Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children

Plasma mannose is suggested to be largely generated from liver glycogen-oriented glucose-6-phosphate. This study examined plasma mannose in glycogen storage disease type Ia (GSD Ia) lacking conversion of glucose-6-phosphate to glucose in the liver. We initially examined fasting—and postprandial 2 h—...

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Veröffentlicht in:	Journal of inherited metabolic disease 2013-01, Vol.36 (1), p.75-81
Hauptverfasser:	Nagasaka, Hironori, Yorifuji, Tohru, Bandsma, Robert H. J., Takatani, Tomozumi, Asano, Hisaki, Mochizuki, Hiroshi, Takuwa, Mayuko, Tsukahara, Hirokazu, Inui, Ayano, Tsunoda, Tomoyuki, Komatsu, Haruki, Hiejima, Eitaro, Fujisawa, Tomoo, Hirano, Ken-ichi, Miida, Takashi, Ohtake, Akira, Taguchi, Tadao, Miwa, Ichitomo
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Biochemistry Biological and medical sciences Blood glucose Blood Glucose - metabolism Carbohydrates Carbohydrates (enzymatic deficiencies). Glycogenosis Child Children Errors of metabolism Fasting Fasting - blood Female General aspects Glucose Glucose - metabolism Glucose-6-phosphate Glucose-6-Phosphate - metabolism Glycogen Glycogen Storage Disease Type I - blood Glycogen Storage Disease Type I - metabolism Human Genetics Humans Insulin Internal Medicine Laboratory testing Lactic acid Lactic Acid - blood Lactic Acid - metabolism Lipids Lipids - blood Liver - metabolism Male Mannose Mannose - blood Medical genetics Medical sciences Medicine Medicine & Public Health Metabolic Diseases Original Article Pediatrics Plasma Postprandial Period - physiology Triglycerides Triglycerides - blood Triglycerides - metabolism
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