Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.3.9 (glucose-6-phosphatase)
 3,081 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 19 patients with a deficiency of glucose-6-phosphatase and 1 patient with a deficiency of glucose-6-phosphate translocase, the effect of nocturnal gastric drip feeding (GDF) on growth and plasma lipids and apolipoproteins was studied. The effect on growth was estimated by determining the height standard deviation score (SDS) of the patients and comparing its changes (delta SDS) over 4-, 2-, and 1-y periods before and 1-, 2-, 5-, and 8-y periods after the institution of GDF. The effect of GDF on plasma lipids and apolipoproteins was investigated by following the concentrations of triglycerides, cholesterol, and apolipoproteins A-I, A-II, B, C-I, C-II, C-III, and E. Growth caught up significantly or remained in the normal range in 14 patients. They were defined as responders to GDF. In the other six patients, growth caught up insufficiently or showed a further deceleration. They were defined as nonresponders to GDF. GDF had only a temporary and marginal effect on plasma lipids and apolipoproteins, but after 5-8 y, the levels of plasma triglycerides, cholesterol, apolipoprotein, B, C-I, C-II, C-III, and E increased further in both responders and nonresponders, whereas apolipoproteins A-I and A-II decreased in nonresponders. There were minor differences in the levels of lipids and apolipoproteins between responders and nonresponders without any discernible trends during the first years of GDF.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Gastric drip feeding in patients with glycogen storage disease type I: its effects on growth and plasma lipids and apolipoproteins. 254 71

Abnormal lipid transport is one of the more severe pathophysiological manifestations of glucose-6-phosphatase deficiency (glycogen storage disease, type I: GSD-I). To characterize further lipoprotein abnormalities in this inborn error of glycogen metabolism, we determined the levels of serum apolipoproteins (Apo) A-I, A-II, B, C-I, C-II, C-III, D, and E in 10 male and 12 female patients, 1-37 yr of age. Results showed that patients with GSD-I have a unique apolipoprotein profile characterized by normal or slightly decreased levels of ApoA-I and ApoA-II, reduced concentrations of ApoD, and significantly increased levels of ApoC-I and ApoC-II (p less than 0.01) and ApoB, ApoC-III, and ApoE (p less than 0.0001) in comparison with age- and sex-matched normolipidemic controls. However, there was some overlap of values in patients and controls for each of the lipid and apolipoprotein constituents with the exception of ApoC-III. This finding supported by the results of the logistic regression analysis showed that the concentration of ApoC-III is the best criterion for distinguishing patients with GSD-I from control subjects and the most characteristic feature of the deranged lipid transport system in this deficiency disease. It remains to be clarified, however, whether the ApoC-III concentrations in patients with GSD-I reflect the degree of other metabolic and clinical manifestations of this disease such as hyperlacticacidemia, hyperuricemia, and growth retardation.
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PMID:The serum apolipoprotein profile of patients with glucose-6-phosphatase deficiency. 385 88

The adipocyte-derived hormone resistin has been proposed as a possible link between obesity and insulin resistance in murine models. Many recent studies have reported physiological roles for resistin in glucose homeostasis, one of which is enhancement of glucose production from the liver by up-regulating gluconeogenic enzymes such as glucose-6-phosphatase and phosphoenolpyruvate carboxykinase. However, its in vivo roles in lipid metabolism still remain to be clarified. In this study, we investigated the effects of resistin overexpression on insulin action and lipid metabolism in C57BL/6 mice using an adenoviral gene transfer technique. Elevated plasma resistin levels in mice treated with the resistin adenovirus (AdmRes) were confirmed by Western blotting analysis and RIAs. Fasting plasma glucose levels did not differ between AdmRes-treated mice and controls, but the basal insulin concentration was significantly elevated in AdmRes-treated mice. In AdmRes-treated mice, the glucose-lowering effect of insulin was impaired, as evaluated by insulin tolerance tests. Furthermore, total cholesterol and triglyceride concentrations were significantly higher, whereas the high-density lipoprotein cholesterol level was significantly lower. Lipoprotein analysis revealed that low-density lipoprotein was markedly increased in AdmRes-treated mice, compared with controls. In addition, in vivo Triton WR-1339 studies showed evidence of enhanced very low-density lipoprotein production in AdmRes-treated mice. The expressions of genes involved in lipoprotein metabolism, such as low-density lipoprotein receptor and apolipoprotein AI in the liver, were decreased. These results suggest that resistin overexpression induces dyslipidemia in mice, which is commonly seen in the insulin-resistant state, partially through enhanced secretion of lipoproteins.
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PMID:Adenovirus-mediated high expression of resistin causes dyslipidemia in mice. 1547 67