3-hydroxy-3-methylglutaryl-CoA
Severe clinical manifestation of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency associated with two novel mutations: a case report
... Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase (mHS) deficiency is an autosomal recessive inborn error of metabolism, which will give rise to failure of ketogenesis in liver ...
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Identification of a Highly Expressed 3 Hydroxy 3 Methylglutaryl CoA Reductase Gene in the Root Tissue of Taraxacum kok saghyz
... The 3-hydroxy-3-methylglutaryl-CoA reductase (HMGR) enzyme catalyzes the conversion of 3-hydroxy-3-methylglutaryl-CoA to meva- lonic acid, a key ...
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Control of 3 Hydroxy 3 Methylglutaryl CoA Reductase Activity in Cultured Human Fibroblasts by Very Low Density Lipoproteins of Subjects with Hypertriglyceridemia
... ultracentrifugation (d < 1.006) or by gel filtration on BioGel A-5m, were about 5 times less effective than LDL in suppressing HMG-CoA reductase activity, based on protein content, in agreement with previous ...
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Effects of the 3 hydroxy 3 methylglutaryl CoA reductase inhibitors, atorvastatin and simvastatin, on the expression of endothelin 1 and endothelial nitric oxide synthase in vascular endothelial cells
... RNA isolation, Northern blotting, and hybridization. Total cellu- lar RNA was isolated from BAEC with the guanidinium thiocyanate- phenol-chloroform method (22) subjected to electrophoresis in 1% agarose gels containing ...
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2. Tingting Tao, Xiaomeng Liu, Jie Chang and Feng Xu
... enzyme 3-hydroxy-3-methylglutaryl-CoA reductase (HMGR) catelyzes the first committed step of isoprenoids biosynthesis in MVA ...
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Identification of 3 methylglutarylcarnitine A new diagnostic metabolite of 3 hydroxy 3 methylglutaryl coenzyme A lyase deficiency
... only 3-methylglutaryl-CoA is a substrate for acylcarnitine ...Neither 3-methylglutaconyl-CoA nor 3-hydroxy-3-methylglutaryl- CoA are excreted as ...of ...
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Energy replacement using glucose does not increase postprandial lipemia after moderate intensity exercise
... A number of studies have shown that TG-rich lipo- proteins, chylomicrons and very-low-density lipoproteins (VLDL), can be bound and also directly internalized by cells via the VLDL receptor, a member of the low-density ...
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56. Inhibition of 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase (ex vivo) by medicinal plants from western ghats
... For the assay of 3-hydroxy-3-methylglutaryl CoA reductase, the incubation mixtures contain 0.5-1.0 mg of microsomal protein, 150 nmoles of HMG- CoA, and 2ìmoles of NADPH. These ...
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Management and long-term evolution of a patient with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency
... In patients with HMG-CoA lyase deficiency the main mechanism that produces fasting hypoglycemia is an excess of glucose use by the peripheral tissues, since these cannot have ketones nor free fatty acids for oxi- ...
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Low density lipoprotein receptor and 3 hydroxy 3 methylglutaryl coenzyme A reductase gene expression in human mononuclear leukocytes is regulated coordinately and parallels gene expression in human liver
... +/- 3 x 10(4)/micrograms of RNA) and mononuclear leukocytes (24 +/- 3 x 10(4)/micrograms of RNA) are indistinguishable, whereas the number of HMG CoA reductase molecules in liver (107 +/- 15 x ...
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MR imaging and proton spectroscopy in 3 hydroxy 3 methylglutaryl coenzyme A lyase deficiency
... HMG CoA lyase deficiency who have survived metabolic crises have remaining deficits, such as developmental delay, microcephaly, and spas- ticity ...case 3 suffered a life-threatening metabolic ...
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3 Hydroxy 3 methylglutaryl coenzyme A reductase in anencephalic and normal human fetal liver
... of 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase, the primary rate- limiting enzyme of cholesterol biosynthesis, in anencephalic liver and normal fetal ...
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Clinical and Biochemical Phenotype in 11 Patients With Mevalonic Aciduria
... The site of the defect in patients with mevalonic aciduria is mevalonate kinase (solid square), following 3-hydroxy- 3-methylglutaryl-coenzyme A (HMG-CoA) reductase, the rate- limiting e[r] ...
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Feedback Regulation of 3 Hydroxy 3 Methylglutaryl Coenzyme A Reductase in Livers of Mice Treated with Mevinolin, a Competitive Inhibitor of the Reductase
... reductase. Despite the abundant amount of cholesterol that was already present in the livers of the mevinolin-treated, cholesterol-fed animals, their elevated HMG CoA reductase could be rapidly suppressed by the ...
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INHIBITION OF 3 HYDROXY 3 METHYLGLUTARYL COENZYME A (HMG Co A) REDUCTASE IN LIVER MICROSOMES BY MORINGA OLEIFERA L POLYPHENOLS
... of 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase, which is the enzyme responsible for conversion of HMG CoA to mevalonate and a rate limiting step in cholesterol ...
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Original Article Effect of atorvastatin, 3-hydroxy-3-methylglutaryl coenzyme a reductase inhibitor (HMG-CoA) on the prevention of osteoporosis in ovariectomized rabbits
... Osteoporosis is a chronic, metabolic, and sys- temic skeletal disorder characterized by dimin- ished bone mass, microarchitectural deteriora- tion, predisposing to decrease bone mineral density (BMD) and increase the ...
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Regulation of acylcoenzyme A Cholesterol acyltransferase and 3 hydroxy 3 methylglutaryl coenzyme A reductase activity by lipoproteins in the intestine of parabiont rats
... (3- hydroxy-3-methylglutaryl coenzyme A [HMG-CoA] reductase activity) and esterification (acylcoenzyme A/cholesterol acyltransferase [ACAT] activity) by lipoproteins and micellar ...
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Suppression of murine neuroblastoma growth in vivo by mevinolin, a competitive inhibitor of 3 hydroxy 3 methylglutaryl coenzyme A reductase
... 3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase catalyzes the formation of mevalonate, an essential precursor for isoprenoid compounds in mammalian cells. Recent studies have ...
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In vivo regulation of human mononuclear leukocyte 3 hydroxy 3 methylglutaryl coenzyme A reductase Studies in normal subjects
... reductase activity within 2 h. In addition, rapid depletion of circulating cholesterol levels by plasmapheresis led to an approximately twofold elevation in enzyme activity within 90 min of treatment. Finally, reductase ...
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Biologically active low density lipoprotein in human peripheral lymph
... of 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMG-CoA reductase), to activate cholesteryl ester synthesis, and to compete with 125I-labeled low density lipoprotein (LDL) for ...
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