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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
115.
Hana Votavová Vladimír Gut Karel Bláha Jaroslav Šponar 《International journal of biological macromolecules》1982,4(6):341-346
DNA complexes with polypeptides (Lys-Ala-Ala)1)] and (Lys-Ala-Ala)34 have been studied using the methods of thermal melting and circular dichroism. Derivative melting curves of (Lys-Ala-Ala)10 DNA differed substantially from those of (Lys-Ala-Ala)34 prepared either by salt gradient dialysis or by direct mixing. Melting curves of the former complex were unimodal or bimodal with Tm increasing continuously withn input lysin-to-DNA phosphate ratio (r); those of the latter complex consisted of three separate transitions with Tm values almost independent of r. Complete reversibility of binding in the (Lys-Ala-Ala)10-DNA system but a slow redistribution of (Lys-Ala-Ala)34 on DNA at low temperature were found in the redistribution experiments Much faster redistribution from denatured to native DNA occurs at the temperature of melting, contributing to the unusual trimodal melting pattern. Circular dichroism curves are very similar for both complexes and indicate little change of DNA conformation upon polypeptide binding. 相似文献
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Chloragogen cells, subserving ion exchange and electron accepting functions, were studied in Tubifex tubifex after insecticide treatment. Chloragogen cells were strongly influenced by in vivo carbofuran poisoning. The first alterations in the chloragogen cells became activated, both the formation and release of the chloragosomes reached a high rate. The released chloragosomes were phagocytosed by the amoebocytes. At an advanced stage of the toxication a heavy loading of the apical cytoplasm of chloragogen cells with lipid droplets, finally degenerative changes both in the chloragogen cells and amoebocytes were observed. Possible mechanisms of the carbofuran toxication and of the protective function of chloragogen cells in T. tubifex are discussed. 相似文献
118.
A. Kotyk J. Horák A. Knotková 《Biochimica et Biophysica Acta (BBA) - Gene Structure and Expression》1982,698(3):243-251
Addition of a metabolizable substrate (glucose, ethanol and, to a degree, trehalose) to non-growing baker's yeast cells causes a boost of protein synthesis, reaching maximum rate 20 min after addition of glucose and 40–50 min after ethanol or trehalose addition. The synthesis involves that of transport proteins for various solutes which appear in the following sequence: H+, l-proline, sulfate, l-leucine, phosphate, α-methyl-d-glucoside, 2-aminoisobutyrate. With the exception of the phosphate transport system, the Kt of the synthesized systems is the same as before stimulation. Glucose is usually the best stimulant, but ethanol matches it in the case of sulfate and exceeds it in the case of proline. This may be connected with ethanol's stimulating the synthesis of transport proteins both in mitochondria and in the cytosol while glucose acts on cytosolic synthesis alone. The stimulation is often repressed by ammonium ions (leucine, proline, sulfate, H+), by antimycin (proline, trehalose, sulfate, H+), by iodoacetamide (all systems tested), and by anaerobic preincubation (leucine, proline, trehalose, sulfate). It is practically absent in a respiration-deficient petite mutant, only little depressed in the op1 mutant lacking ADP/ATP exchange in mitochondria, but totally suppressed (with the exception of transport of phosphate) in a low-phosphorus strain. The addition of glucose causes a drop in intracellular inorganic monophosphate by 30%, diphosphate by 45%, ATP by 70%, in total amino acids by nearly 50%, in transmembrane potential (absolute value) by about 50%, an increase of high-molecular-weight polyphosphate by 65%, of total cAMP by more than 100%, in the endogenous respiration rate by more than 100%, and a change of intracellular pH from 6.80 to 7.05. Ethanol caused practically no change in ATP, total amino acids, endogenous respiration, intracellular pH or transmembrane potential; a slight decrease in inorganic monophosphate and diphosphate and a sizeable increase in high-molecular-weight polyphosphate. The synthesis of the various transport proteins thus appears to draw its energy from different sources and with different susceptibility to inhibitors. It is much more stimulated in facultatively aerobic species (Saccharomyces cerevisiae, Endomyces magnusii) than in strictly aerobic ones (Rhodotorula glutinis, Candida parapsilosis) where an inhibition of transport activity is often observed after preincubation with metabolizable substrates. 相似文献
119.
HAROLD St. JOHN F.L.S. 《Biological journal of the Linnean Society. Linnean Society of London》1972,4(4):305-310
Sydney Parkinson, aged 18, was Sir Joseph Banks' private assistant and artist on Captain Cook's first voyage around the world. Parkinson died on the voyage. Among his effects delivered to his brother Stanfield were drawings and notes which the latter published as a journal. Included in it is a chapter on the economic plants of Tahiti, and most of them are given scientific names, some of which were new. In 1941 Fosberg adopted two of them to form names which were new combinations under Artocarpus , and Inocarpus . The writer now indicates that Parkinson's names of 1773 were mononomials, hence invalid, but that in the German edition of 1774, edited by Herr "Z", the names are valid binomials. For the two plants mentioned, the authorship of the combinations is revised. 相似文献
120.