期刊
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
卷 86, 期 8, 页码 3724-3728出版社
ENDOCRINE SOC
DOI: 10.1210/jc.86.8.3724
关键词
-
资金
- NCRR NIH HHS [M01-RR-00240, M01 RR000240] Funding Source: Medline
- NIDDK NIH HHS [T32 DK007314, F32 DK009985, R01-DK-56268, R01 DK056268, F32 DK009985-02, T32-DK-07314] Funding Source: Medline
- PHS HHS [R01-53012] Funding Source: Medline
Mutations of glutamate dehydrogenase cause the hyperinsulinism/hyperammonemia syndrome by desensitizing glutamate dehydrogenase to allosteric inhibition by GTP. Normal allosteric activation of glutamate dehydrogenase by leucine is thus uninhibited, leading us to propose that children with hyperinsulinism/hyperammonemia syndrome will have exaggerated acute insulin responses to leucine in the postabsorptive state. As hyperglycemia increases beta -cell GTP, we also postulated that high glucose concentrations would extinguish abnormal responsiveness to leucine in hyperinsulinism/hyperammonemia syndrome patients. After an overnight fast, seven hyperinsulinism/hyperammonemia syndrome patients (aged 9 months to 29 yr) had acute insulin responses to leucine performed using an iv bolus Of L-leucine (15 mg/kg) administered over I min and plasma insulin measurements obtained at -10, -5, 0, 1, 3, and 5 min. The acute insulin response to leucine was defined as the mean increase in insulin from baseline at I and 3 min after an iv leucine bolus. The hyperinsulinism/hyperammonemia syndrome group had excessively increased insulin responses to leucine (mean +/- SEM, 73 +/- 21 mu IU/ml) compared with the control children and adults (n = 17) who had no response to leucine (1.9 +/- 2.7 muU/ml; P < 0.05). Four hyperinsulinism/hyperammonemia syndrome patients then had acute insulin responses to leucine repeated at hyperglycemia (blood glucose, 150-180 mg/dl). High blood glucose suppressed their abnormal baseline acute insulin responses to leucine of 180,98,47, and 28 muU/ml to 73,0,6, and 19 muU/ml, respectively. This suppression suggests that protein-induced hypoglycemia in hyperinsulinism/hyperammonemia syndrome patients may be prevented by carbohydrate loading before protein consumption.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据