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Summary The child with iminoglycinuria is in our observation. Hyperprolinuria was seen at 5 months by screening program. The child was repeatedly examined in the hospital and was seen last time at 16 months. IQ was 67, in the urine were excessive amounts of glycine, proline a hydroxyproline. In the blood aminoacids were in normal levels. In the child was noticed the same increase of proline in the blood as in the control child of the same age following loading test with L-proline, indicating normal intestinal absoption. Both parents and father's sister's 2-year-old mentally retarded child exhibit excessive glycinuria. The father, his sister, father's father, and grandfather are partialy, congenitaly deaf.  相似文献   
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Small molecule inhibitors have a powerful blocking action on viral polymerases. The bioavailability of the inhibitor, nevertheless, often raise a significant selectivity constraint and may substantially limit the efficacy of therapy. Phosphonoacetic acid has long been known to possess a restricted potential to block DNA biosynthesis. In order to achieve a better affinity, this compound has been linked with natural nucleotide at different positions. The structural context of the resulted conjugates has been found to be crucial for the acquisition by DNA polymerases. We show that nucleobase-conjugated phosphonoacetic acid is being accepted, but this alters the processivity of DNA polymerases. The data presented here not only provide a mechanistic rationale for a switch in the mode of DNA synthesis, but also highlight the nucleobase-targeted nucleotide functionalization as a route for enhancing the specificity of small molecule inhibitors.  相似文献   
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Six thousand anthers ofSecale cereale cv. Dańkowskie Zlote and inbred lines L214 L215 and L258c/5, in the uninuclear microspore were cultured over a period of three months on Murashige and Skoog nutrient medium supplemented with IAA (1–2μg1−1), kinetin (0.2 to (1.0μg1−1) and 2,4-D (0.5–2.0μg1−1). First divisions of microspore were observed after 7 days. In the course of 10 weeks, 4 albino tic embryos at the cotyledon stage were observed that died away in the course of further culture. The course of androgenesis was regular in inbred lines and irregular in rye cv. Dańkowskie Ziote. The efficiency of androgenesis and the amount of observed globular structures in anthers were also dependent on the genetic potency of the material. Inbred lines did not show any greater viability of embryos.  相似文献   
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Summary Kinetic parameters of production of clavine alkaloids were evaluated in twoClaviceps purpurea strains. Mutagenesis brought about enhanced resistance of the biosynthetic system towards alkaloids. Addition of glucose into the fermentation medium altered the zero order kinetics of production to activation-inhibition kinetics. The glucose treatment allowed performance of both elymoclavine-inhibitionless and clavine alkaloid-decompositionless fermentations if a combination of fermentation and separation units in a closed loop was used.Nomenlacture k 1 rate constant of agroclavine synthesis (mg Agro · mg Elymo/l·g DW·day for stage 1, mg Agro/g DW·day for stage 2) - k 2 parameter describing inhibition of agroclavine formation rate by elymoclavine (mg Elymo/l) - k 3 specific rate of agroclavine decay (l/g DW·day) - k 4 maximal specific rate of elymoclavine synthesis (stage 1, 1/g DW·day, stage 2, mg Elymo/g DW·day) - k 4 maximal specific rate of elymoclavine synthesis in stage 1 (inhibition-activation mechanism) (mg Elymo/g DW·day) - k 5 physiological constant describing the elymoclavine decay rate (l2/g DW·day·mg Elymo) - k 5 physiological constant describing the activation of elymoclavine biosynthesis by elymoclavine (mg Elymo/l) - k 6 physiological constant describing the repression of elymoclavine biosynthesis by elymoclavine (mg Elymo/l) - k 7 maximal specific growth rate (1/day) - k 8 specific rate of biomass decay (l/g DW·day) - A agroclavine concentration (mg/l) - E elymoclavine concentration (mg/l) - r A specific rate of agroclavine biosynthesis (mg Agro/g DW·day) - r E specific rate of elymoclavine biosynthesis (mg Elymo/g DW·day) - r i specific rate of alkaloid biosynthesis (mg alkaloid/g DW·day) - X dry biomass concentration (g/l) - specific growth rate (1/day) Abbreviations Agro agroclavine - Elymo elymoclavine - Chano chanoclavine - DW dry weight of biomass  相似文献   
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Summary The effect was studied of the method of drying chymotrypsin attached to bead cellulose on its chemical and physical characteristics. These characteristics are not deteriorated when replacing the commonly used lyophilisation by fluid drying in the air stream. The preparations saved essentially the same proteolytic activity as well as stability even when increasing the drying air temperature to 70°C.  相似文献   
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Sterol composition was examined during batch growth on complex media containing ethanol, molasses or glucose as the carbon source. The molasses-grown cells exhibited a balanced sterol composition throughout growth, maintaining the proportion of ergosterol to 24:28-dehydroergosterol equal to 1.4. The negative effect of glucose on sterol synthesis manifested itself by decreasing the accumulation of 24:28-dehydroergosterol and total sterols but not of ergosterol. Using ethanol as the sole carbon source, a large amount of 24:28-dehydroergosterol accumulated, partly at the expense of other sterols. The gradual addition of nitrogen source during growth significantly decreased the accumulation of ergosterol, 24:28-dehydroergosterol and of total sterols. A general scheme of regulation of sterol synthesis in baker's yeast is presented.  相似文献   
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