A STUDY OF THE DOSING AND EFFICACY OF MODIFIED RESISTANT CORNSTARCH IN GSD 1A PA

改性抗性玉米淀粉在 GSD 1A PA 中的用量和功效研究

• 批准号: 7950763
• 负责人: David Daniel Weinstein
• 金额: $ 1.33万
• 依托单位: UNIVERSITY OF FLORIDA
• 依托单位国家: 美国
• 财政年份: 2008
• 资助国家: 美国
• 起止时间: 2008-12-01 至 2009-07-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7950763
• 关键词: Amylases; Biochemical; Clinical Research; Computer Retrieval of Information on Scientific Projects Database; Corn starch preparation; Dose; Enzymes; Funding; Gluconeogenesis; Glucose; Glycemic Index; Glycogen; Glycogen Storage Disease; Glycogen Storage Disease Type I; Grant; Heating; Hepatic; Hereditary Disease; Hypoglycemia; Institution; Kidney; Liver; Maintenance; Modification; Patients; Phenotype; Postabsorptive Hypoglycemia; Research; Research Personnel; Resistance; Resources; Source; Starch; United States National Institutes of Health; blood glucose regulation; glucose-6-phosphatase; glycogenolysis; improved

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The glycogen storage diseases GSD are a set of genetic disorders in which the phenotype is severe hypoglycemia because of the inability to produce glucose from glycogen in the liver and kidney. Type I glycogen storage disease GSD1 is caused by a deficiency of hepatic glucose-6-phosphatase, which is an integral part of both glycogenolysis and gluconeogenesis. Lack of this enzyme results in the inability to maintain normal glucose homeostasis and severe fasting hypoglycemia. Patients presently are treated with an average of 6.2 doses of cornstarch per day. There is a need for a digestible starch that can provide a source of glucose for longer duration after meals in GSD patients. Vitastarch is a high amylase, heat modified waxy cornstarch which recently was found to have a lower glycemic index and be longer lasting when compared with traditional cornstarch in glycogen storage disease types 1a and 1b. A study is now proposed to determine the optimal dosing of this new product and to determine if modification of the amount of the new product will offer biochemical benefit and improved maintenance of glucose concentrations in patients with GSD 1a.

该子项目是利用该技术的众多研究子项目之一 资源由 NIH/NCRR 资助的中心拨款提供。子项目和 研究者 (PI) 可能已从 NIH 的另一个来源获得主要资金， 因此可以在其他 CRISP 条目中表示。列出的机构是 对于中心来说，它不一定是研究者的机构。 糖原贮积症GSD是一组遗传性疾病，其表型为严重低血糖，因为肝脏和肾脏无法从糖原产生葡萄糖。 I 型糖原贮积症 GSD1 是由肝葡萄糖 6 磷酸酶缺乏引起的，肝葡萄糖 6 磷酸酶是糖原分解和糖异生的重要组成部分。 缺乏这种酶会导致无法维持正常的葡萄糖稳态和严重的空腹低血糖。 目前患者平均每天接受 6.2 剂量的玉米淀粉治疗。 需要一种可消化的淀粉来为 GSD 患者提供餐后较长时间的葡萄糖来源。 Vitastarch 是一种高淀粉酶、热改性蜡质玉米淀粉，最近发现与传统玉米淀粉相比，它在治疗 1a 型和 1b 型糖原贮积症方面具有较低的血糖指数和更持久的效果。 现在提议进行一项研究，以确定该新产品的最佳剂量，并确定新产品用量的修改是否会为 GSD 1a 患者带来生化益处并改善葡萄糖浓度的维持。