Results 211 to 220 of about 30,985 (250)
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Mevalonate kinase deficiency in a dizygotic twin with mild mevalonic aciduria
Journal of Inherited Metabolic Disease, 1997Mevalonic aciduria, the result of mevalonate kinase (MKase) deficiency (McKusick 251170), is a rare abnormality of cholesterol and nonsterol isoprene biosynthesis identified in approximately 14 patients. The phenotype includes developmental delays, failure to thrive, hypotonia, ataxia, organomegaly, dysmorphia, cataracts, lymphadenopathy, myopathy and ...
K M, Gibson +3 more
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Bifunctional Inhibitors of Mevalonate Kinase and Mevalonate 5-Diphosphate Decarboxylase
Organic Letters, 2006[structure: see text] A bifunctional inhibitor of mevalonate kinase and mevalonate 5-diphosphate decarboxylase was synthesized. Both enzymes are in the cholesterol biosynthetic pathway and play an important role in regulating cholesterol biosynthesis.
Yongge, Qiu, Ding, Li
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Mevalonate Kinase Deficiency: Disclosing the Role of Mevalonate Pathway Modulation in Inflammation
Current Pharmaceutical Design, 2012Inflammation is a highly regulated process involved both in the response to pathogens as well as in tissue homeostasis. In recent years, a complex network of proteins in charge of inflammation control has been revealed by the study of hereditary periodic fever syndromes.
A. Marcuzzi +5 more
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Severe phenotypic spectrum of mevalonate kinase deficiency with minimal mevalonic aciduria
Molecular Genetics and Metabolism, 2012Mevalonate kinase deficiency is a rare autosomal recessively inherited organic aciduria with a complex multi-systemic phenotype. We describe two deceased patients with clinically severe mevalonate kinase (MK) deficiency confirmed by MK mutation analysis.
Chitra, Prasad +2 more
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Genes for mevalonate biosynthesis in Phycomyces
Molecular Genetics and Genomics, 2002Terpenoids or isoprenoids constitute a vast family of organic compounds that includes sterols and carotenoids. The terpenoids in many organisms share early steps in their biosynthesis, including the synthesis of 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) and its conversion to mevalonate.
J, Ruiz-Albert +2 more
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Metabolic Engineering of the Mevalonate and Non-Mevalonate Pathways in Tomato
2003In higher plants isoprenoids have many essential roles e.g. in membrane structure (phytosterols), redox chemistry (plastoquinone) and as antioxidants (carotenoids). Despite their functional and chemical diversity all isoprenoids are biosynthetically related from a common precursor, isopentenyl pyrophosphate (IPP).
E. M. Enfissi +5 more
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Mevalonate synthesis in the mitochondria of yeast
Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1973Abstract 1. 1. Subcellular fractions were obtained bydifferential centrifugation of the homogenate of spheroplasts prepared from aerobically cultured Saccharomyces cerevisiae. The localization of the enzymes involved in the early stage of ergosterol synthesis was investigated. 2. 2.
I, Shimizu +3 more
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Multienzyme mevalonate pathway bioreactor
Biotechnology and Bioengineering, 2004AbstractThe five‐carbon metabolic intermediate isopentenyl diphosphate constitutes the basic building block for the biosynthesis of all isoprenoids in all forms of life. Two distinct pathways lead from amphibolic intermediates to isopentenyl diphosphate.
Autumn, Sutherlin, Victor W, Rodwell
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