지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2011.1
- 수록면
- 263 - 267 (5page)
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초록· 키워드
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Purpose: Mucopolysaccharidosis II (MPS II) is a lysosomal storage disorder caused by a deficiency of iduronate-2 sulfatase (IdS), which is involved in the degradation of glycosaminoglycan (GAG). In this study, the frequency of fasting hypoglycemia in patients with MPS II was investigated and changes in accumulation of glycogen and GAG in the hepatocytes of IdS-knockout (KO) mice were evaluated before and after recombinant IdS enzyme replacement therapy (ERT). Materials and Methods: Plasma glucose levels were evaluated after an 8-hour fast in 50 patients with MPS II. The IdS-KO mice were divided into three groups (group 2; saline, group 3; 0.15 mg/kg of IdS, and group 4; 0.5 mg/kg of IdS); wild-type mice were included as controls (group 1). ERT was initiated intravenously at four weeks of age, and continued every week until 20 weeks of age. Results: The mean glucose level after an 8-hour fast was 94.1 ± 23.7 mg/dL in the patients with MPS II. Two (4%) out of 50 patients had fasting hypoglycemia. For the mice, GAG in the lysosomes nearly disappeared and glycogen particles in the cytoplasm were restored to the normal range in group 4. Conclusion: Glucose metabolism in patients with MPS II appeared to function well despite hepatocytic GAG accumulation and hypothetical glycogen depletion. A higher dose of IdS infusion in MPS II mice led to disappearance of lysosomal GAG and restoration of glycogen to the cytoplasm of hepatocytes.
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참고문헌 (10)
참고문헌 신청
Garcia AR / 2007 / Preclinical dose ranging studies for enzyme replacement therapy with idursulfase in a knock-out mouse model of MPS II / Mol Genet Metab 91 : 183 ~ 190
Huidekoper HH / 2008 / Fasting adaptation in idiopathic ketotic hypoglycemia: a mismatch between glucose production and demand / Eur J Pediatr 167 : 859 ~ 865
Muenzer J / 2002 / Enzyme replacement therapy in mucopolysaccharidosis type II (Hunter syndrome): a preliminary report / Acta Paediatr Suppl 91 : 98 ~ 99
Muenzer J / 2006 / A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome) / Genet Med 8 : 465 ~ 473
Muenzer J / 2007 / A phase I/II clinical trial of enzyme replacement therapy in mucopolysaccharidosis II (Hunter syndrome) / Mol Genet Metab 90 : 329 ~ 337
Huidekoper HH / 2008 / Fasting adaptation in idiopathic ketotic hypoglycemia: a mismatch between glucose production and demand / Eur J Pediatr 167 : 859 ~ 865
Muenzer J / 2002 / Enzyme replacement therapy in mucopolysaccharidosis type II (Hunter syndrome): a preliminary report / Acta Paediatr Suppl 91 : 98 ~ 99
Muenzer J / 2006 / A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome) / Genet Med 8 : 465 ~ 473
Muenzer J / 2007 / A phase I/II clinical trial of enzyme replacement therapy in mucopolysaccharidosis II (Hunter syndrome) / Mol Genet Metab 90 : 329 ~ 337
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