Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: CAS:1069-31-4 (ornithine)
 6,755 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An amino acid transport defect which occurs in the erythrocytes of adult sheep is also present in foetal erythrocytes from newborn lambs which have inherited the lesion. The transport defect in erythrocytes from adult sheep is associated with high intracellular levels of ornithine and lysine and a markedly diminished GSH concentration. Although the lesion in foetal cells also results in the accumulation of ornithine and lysine, the intracellular GSH concentration is only moderately diminished.
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PMID:Amino acid transport properties of erythrocytes from normal newborn lambs and lambs with an inherited defect in amino acid transport. 68 27

L-Lysine-alpha-ketoglutarate reductase (N5-(1,3-dicarboxypropyl)-L-lysine: NADP+ oxidoreductase (L-lysine-forming, EC 1.5.1.8) was purified from rat liver mitochondria to a homogeneous state judged by SDS polyacrylamide gel electrophoresis, and its molecular weight was estimated as 52000. On Sepharose 4B filtration it has a molecular weight of 230 000 and it is suggested that the active enzyme is a tetramer of subunits of similar size. The purified enzyme was clearly separated from saccharopine dehydrogenase (N5-(1,3-dicarboxypropyl)-L-lysine:NAD+ oxidoreductase (L-glutamate-forming, EC 1.5.1.9). The reactions of purified L-lysine-alpha-ketoglutarate reductase favored the forward reaction (saccharopine formation) and the rate of the reverse reaction (lysine formation) was only 3--5% that of the forward reaction. The forward reaction was specific for L-lysine, alpha-ketoglutarate and NADPH and followed Michaelis-Menten kinetics, whereas the dose vs. response curve of the reverse reaction was sigmoidal with saccharopine. Among the amino acids examined, ornithine, leucine and tryptophan inhibited the forward reaction competitively. These results are different from earlier reports on human and yeast enzymes. The fact that rats fed on lysine-deficient diet do not lose weight much is discussed in relation to the properties of this enzyme.
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PMID:Purification and properties of L-lysine-alpha-ketoglutarate reductase from rat liver mitochondria. 68 35

Cistin-lisinuria is not a unfrequent etiology of lithiasis in children. Six patients have been studied as well as their families, being a total of 45 patients. Lithiasis was present in ten of our patients, and hyperaminoaciduria, without lithiasis in ten, affecting in both cases (with or without litiasis) either all the four amynoacide (cystine, lysine, arginine and ornithine) or only one or two of them. Authors have not found any correlation between clearance of cystine and the presence or absence of lithiasis; on the other hand the best index to correlate the presence of lithiasis is to reach a value, equal or superior to 300 mgr of cystine excreted in the urine per 1 gr of creatinine excreted in urine. The correct treatment in cystinuric patients with lithiasis is to alcalize the urine maintaining a constant urinary pH between 7-8, giving a sufficient dose of sodium bicarbonate per os. In case of resistance to this treatment or if a great calculi is present, D-penicillamine would be an efficient treatment to disolve the calculi. These treatment when carried out under analytical control presents no problems or complications in their experience.
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PMID:[Cystine-lysinuric lithiasis]. 69 18

Heparin uptake and desulphation by cultured macrophages were investigated. Histones, polyamino-acids, protamine and eosinophil-basic protein stimulated both heparin uptake and desulphation, processes found to be non-related. Poly-L-ornithine and poly-DL-lysine increased the heparin uptake by about 33-fold, and histone produced up to 7.5-fold increase in the desulphation. The same polycations inhibited heparin desulphation by macrophage extracts.
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PMID:Increased uptake and desulphation of heparin by mouse macrophages in the presence of polycations. 71 98

In chronic uremia patients on an 18-g protein diet for 2 to 3 weeks, the intracellular free amino acid pattern in muscle was grossly abnormal, with low concentrations of threonine, valine, carnosine, and high concentrations of aspartate, glutamine, glycine, alanine, and taurine. When the protein-poor diet was supplemented with essential amino acids (2 to 3 times that for minimum requirements) and histidine, these abnormalities were partly corrected. Other abnormalities, however, were observed, such as low intracellular concentrations of phenylalanine and tyrosine, and high concentrations of lysine, histidine, ornithine, and citrulline.
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PMID:Effect of essential amino acid supplementation on muscle and plasma free amino acids in chronic uremia. 73 92

A renal calculus composed principally of the amino acid, cystine, was found in an 8-year-old male maned wolf (Chrysocyon brachyurus). Cystine crystals were found in the urine sediment. The renal clearance of 10 amino acids was abnormal, whereas reabsorption of others was normal. The renal clearance of cystine, lysine, ornithine, and arginine exceeded the filtered load. The renal tubular handling of glucose, phosphate, sodium, potassium, and uric acid was identical to that for the clinically normal dog. These findings indicated an isolated renal tubular defect for cystine and other amino acids.
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PMID:Cystinuria in a maned wolf. 73 37

A metabolic study in a case of hyperornithinemia with gyrate atrophy of choroid and retina is presented. It is demonstrated that the lysine-ornithine antagonism is a physiological, safe, easy to handle therapeutic tool in hyperornithinemia. A lysine load was invariably associated with a reduction of plasma ornithine concentration. This happened at any level of protein intake. It was also shown that diets containing a low protein intake are invariably associated with negative nitrogen balance.
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PMID:Lysine in treatment of hyperornithinemia. 74 43

The content of free amino acids is determined in tissue of rats which prefer either water or 5% water solution of ethanol. The rats which prefer ethanol have a higher content of taurine, threonine and urea in the liver, a larger amount of lysine and beta-aminobutyrate in the brain, less of ornithine in blood plasm and a larger amount of taurine, aspartate and urea in urine. Significant differences are supposed to be in the amino acids metabolism in rats which prefer either water or ethanol.
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PMID:[Free amino acid content of tissues of rats which prefer either water or ethanol solutions]. 74 96

With the aid of ion exchange column chromatography we determined quantitatively the free amino acids in eccrine, thermal sweat. Sweat was collected (a) from the face of 27 healthy men and 26 healthy women (b) from the face, chest, armpits, shoulders, back, upper part of the abdomen, hypogastrium, forearms and thighs of the same individual and (c) from the face of the same individual at different times. Sweat was deproteinised by adding an equal volume of 5% sulphosalicylic acid. 1. The results showed not only a constant, qualitative amino acid pattern in sweat, but also a relative constancy among the individual amino acids. 2. The concentrations of the free amino acids in sweat showed significant, individual variations. Particularly high excretion rates were observed in the following amino acids: alanine, glycine, citrulline, histidine, ornithine, threonine and serine. 3. As compared to men, women had an increased excretion of all the examined amino acids in sweat from the face, except for cystine. Statistically significant higher excretion was seen within this sex-specific comparison for the following amino acids: alanine, citrulline, glycine, histidine, isoleucine, serine, taurine, threonine, tyrosine and valine. 4. Essential amino acids such as isoleucine, leucine, lysine, methionine, phenylalanine and valine and also cystine were always excreted only in small amounts. 5. Significant differences were also observed in the total amino acid excretion and in the individual amino acid excretions in sweat obtained from different parts of the body of the same person. 6. The amino acid concentrations determined in the sweat from the face of the same individual at different times showed a relative constancy as compared to the large differences of the amino acid concentrations determined in the sweat from the face of different individuals.
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PMID:[Free amino acids in human Eocrine sweat (author's transl)]. 76 10

About 10 amino derivatives, capable to inhibit agmatinase of Proteus vulgaris, are found. Hexamethylenediamine was found to possess the highest inhibitory effect among diamines, the inhibitory constant being 2.5 mM. The substitution of an amino group for methyl one in a diamine increased its inhibitory effect. The presence of carboxyl group in the molecule of a reagent resulted in a complete elimination of the inhibitory effect (putrescine-ornithine and cadaverine-lysine pairs were tested). The degree of inhibition was found to be pH-dependent. A supposition is made on hydrophobic character of substrate binding by the enzyme.
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PMID:[Inhibition of bacterial agmatinase by several aliphatic diamines]. 77 36


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