Recent advances of clinical and genomic study of glycogen storage disease type ⅲ

Clinical and Mutation Analysis of Glycogen Storage Disease Type ⅲ

糖原累积症-Ⅲ型的临床和糖原脱枝酶基因突变分析

The clinical and pathological features of Chinese patients of type ⅰ glycogen storage disease with renal involvement

Ⅰ型糖原贮积症肾损害临床表现和病理改变

Objective: To study the relationship between hepatocyte endoplasmic reticulum, glycogen granule and lipid droplet morphological variety and time with mice following SeO 2 poisoning.

目的：对SeO2中毒所致肝细胞内质网、糖原和脂滴的变化进行时间形态学定量研究。

Results The average proportion of RRF was 5.3% in 4 cases, and glycogen granule deposition and crystal include bodies in 2 cases.

6例患者中4例发现破碎红纤维（RRF），其平均比例为5.3%；超微结构观察有线粒体异常及糖原颗粒沉积，其中有2例发现有典型晶格状包涵体。

The results showed that induced HPP colonies cells with a specific ultrastructure similar to hepatic epithelial cells, such as many microvilli on the cell surface, large nucleolus and many round-shaped mitochondria, and lots of glycogen granule in the cytoplasm.

检测结果显示诱导后的部分细胞具有肝细胞特异性的超微结构，在细胞表面形成微绒毛，在胞浆内具有较大的细胞核，大量的圆形线粒体和糖原颗粒。

Effect of dietary carbohydrate levels on growth, feed utilization and liver glycogen in Chinese mitten-handed crab Eriocheir sinensis Carbohydrate, fat and protein are the basic feedstuffs.

饲料不同碳水化合物水平对中华绒螯蟹生长、饲料利用及肝糖原含量的影响碳水化合物、脂肪与蛋白质都是饲料中的基本营养成分。

Variation of animal's liver glycogen contents induced by noise

取肝脏检测肝糖原。噪声引起动物肝糖原变化

Meanwhile, the function evaluations of by the blood sugar test, liver glycogen test and swimming trial showed that saccharide mixtures could enhance anti-fatigue function.

同时，以血糖、肝糖原和游泳时间为指标的功能性评价表明复配糖类有增加抗疲劳的功能。

Liver glycogen contents in model mice significantly declined except neonatal STZ-induced model mice.

除n5STZ诱导模型外，其它模型组肝糖原的含量均明显低于对照组。

Capability of the astragals to prevent damage of animal's liver glycogen from noise

黄芪对噪声引起动物肝糖原损害的抵抗能力

Carbohydrates were first named according to their natural sources; e.g., beet sugar, cane sugar, grape sugar, malt sugar, milk sugar, cornstarch, liver glycogen, and sweet corn glycogen.

碳水化合物最初是按照它们的天然来源来命名的，例如甜菜糖、甘蔗糖、葡萄糖、麦芽糖、乳糖、玉米淀粉、肝糖原、甜玉米糖原。

Influence of Several Vitamins on the Mice Liver Glycogen Content and the Mice Liver GPT Activity Caused by Noise Exposure

几种维生素对噪声暴露小鼠肝脏糖原含量和GPT活性变化的影响

Model group in liver glycogen content was significantly decreased compared with the control group ( P0.01); prevention of hepatic glycogen in rats was also lower than the control group ( P0.05), compared with model group, but there was no statistically significant.

肝糖原含量预防组与对照组相比明显下降（P0.01），预防组大鼠肝糖原含量也低于对照组（P0.05），比模型组高，但无统计学意义。

The contents of hemoglobin, blood lactic acid, serum urea nitrogen, muscle glycogen, and liver glycogen of various group mice were detected after resting30min.

小鼠处死后，取骨骼肌和肝脏，测定肌糖原和肝糖原的含量。

However, TMP reduced the content of liver glycogen, plasma lactic acid, and inhibited the activity of lactic dehydrogenase.

与此同时，金耳菌丝体多糖可明显降低SD大鼠的肝糖元和血液乳酸含量，抑制乳酸脱氢酶活力。

Carnosine supplementation decreased the production of serum lactic acid, serum urea, muscle MDA, and the consumption of liver glycogen, muscle GSH and SOD.

小鼠运动后产生的乳酸量、肝糖原消耗量、血清尿素氮、肌肉组织SOD，GSH消耗量和MDA产生量，与对照组相比均有不同程度减少；

Results: Compared with the control group, both the burdened swimming time and the liver glycogen level of 8.4 、 12.6mg/ ( kg bw · d) groups increased significantly ( P < 0.05).

结果：中、高剂量组的负重游泳时间和肝糖原含量均显著高于对照组（P<0.05）；

CONCLUSION: Diabetic subjects show impaired glycogen metabolism with the diminished liver glycogen and muscle glycogen contents, which indicates the key role of glycogen metabolism in the pathogenesis of diabetes.

结论：糖尿病动物模型出现糖原代谢异常，表现为肝糖原和肌糖原减少，提示糖原代谢在糖尿病发病过程中起着重要的作用。

RESULTS: Significant decreases in blood glucose and liver glycogen levels were induced in a dose-dependent manner after administration of GGP 50, 100, or 200 mg/ kg injected ip or sc to normal mice and injected im 30 or 60 mg/ kg to normal rabbits.

结果：给正常小鼠ip或sc人参糖肽（50100和200mg/kg）或按30和60mg/g剂量给正常家兔im人参糖肽，能明显降低正常动物的血糖和肝糖元，并且有剂量依赖关系；

Moreover, the content of liver glycogen and activity of muscle ATPase of rats were increased while the level of cerebral lactate was decreased ( P < 0.05, P < 0.01) in mixture supplemented group.

此外，合剂还可提高模拟晕船刺激后大鼠肝糖原含量，降低脑乳酸浓度，提高肌肉ATP酶活性（P<0.05，P<0.01）。