• Childhood Kidney Diseases
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• v.27 no.2
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• pp.127-132
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• 2023

Cystinuria, a genetically inherited disorder, is a rare cause of kidney stones. It is characterized by impaired transport of cystine and amino acids in the proximal renal tubule and the small intestine. Most patients develop cystine stones throughout their lifetime. Recurrent renal stones need to be extracted by repeated urologic interventions. Treatment options of cystinuria for preventing stone recurrence are limited and poorly tolerated. In this study, we report a pediatric case of cystinuria with a heterozygous SLC3A1 mutation diagnosed by stone analysis, measurement of urine cystine excretion, and genetic analysis. There were recurrent renal stones despite repetitive shock wave lithotripsy and retrograde intrarenal surgery. However, the rate of stone formation seemed to be slower after D-penicillamine was added into adequate hydration and urinary alkalinization.

• Journal of Plant Biology
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• v.17 no.1
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• pp.9-14
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• 1974

Inhibitory effect of alantolactone and isoalantolactone was shwon in Avena straight growth test and in the formation of adventitious root in Phaseolus seedling. However, di-, and tetrahydroalantolactones given no effect on the elongation and the rooting. Inhibitory effect of alantolactone could partly be removed by cysteine, cystine, and reduced glutthione. The plant materials were made less sensitive to alantolactone by the pretreatment of cysteine, but cysteine supplied after the treatment of alantolactone brought about no effect on the action of alantolactone. A new spot was shown on TLC plate from the mixture of alantolactone and cysteine, indicating that alantolactone can be inactivated by cysteine, not cystine, without any biological processes.

• Journal of the Korean Society of Food Science and Nutrition
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• v.11 no.3
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• pp.37-40
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• 1982

Fifteen free amino acids except tryptophan, proline and cystine were identified from Korean red ginseng and dried ginsengs from Korea, America and Canada using by high performance liquid chromatography (HPLC). Arginine was 72.6% of total free amino acids in the red ginseng and 48.2 to 68.7% in the dried ginsengs. The content of each free amino acid was lower in the red ginseng than in Korean dried ginseng. Most free amino acids in Korean dried ginseng showed higher content than those in American and Canadian ones. Tryptophan, proline cystine, methionine and phenylalanine were not detected in the extracts of red ginseng and of Korean white ginseng. Arginine was highest in these extracts and all free amino acids were higher in the white ginseng extract.

• Applied Biological Chemistry
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• v.9
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• pp.59-64
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• 1968

Takju (Korean native Sake) was mashed with two different materials. One of the material was polished rice and Nuruk(mold wheat), the other one was corn and Nuruk. The amino acids in those fermenting mashes were identified by paper partition chromatography at regular intervals. The results were summarized as follows; a) Following 14 kinds of amino acids were identified in the mash of rice material; lysine, valine, proline, leucine, serine, glycine, aspartic acid, alanine, cystine, tyrosine, histidine, glutamic acid, tryptophan and phenylalanine b) Following 12 kinds of amino acids were identified in the mash of corn material; lysine, valine, proline, leucine, serine, glycine, aspartic acid, alanine, cystine, histidine, argrinine and tryptophan. c) The main amino acids in the Takju mash according to the color density of the each amino acid spot on the paper chromatograms were checked as lysine, valine, leucine, serine, proline and glycine.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.4 no.1
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• pp.23-29
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• 2004

Cystinosis, an autosomal recessively inherited lysosomal storage disease, results from impaired transport of the amino acid cystine out of cellular lysosomes. The consequent accumulation and crystallization of cystine destroys tissues, causing growth retardation, Fanconi syndrome, renal failure, eye problems, and endocrinopathies. The gene for cystinosis, CTNS, was mapped to chromosome 17p13. The diagnosis of cystinosis is made by measuring the leukocyte cystine content and the presence of typical corneal crystals on slit-lamp examination is also diagnostic. Since treatment with cysteamine has proved extremely effective, early diagnosis and treatment are critical aspects. We experienced a typical case of cystinosis in a 12-year-old boy with growth retardation.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.12 no.4
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• pp.235-240
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• 1979

A study on the amino acid composition of raw frozen krill, and krill solubles manufactured in forms of paste and powder has been carried out. The raw frozen krill was thawed, chopped, mixed and homogenized with same amount of water. The mixture was autolyzed or hydrolyzed by tile addition of $0.2\%$ pronase-p, a commercial proteolytic enzyme, to the weight of the raw frozen krill at $45^{\circ}C$ for 4 hours. After a thermal inactivation of enzymes at $95^{\circ}C$ for 15 minutes, the autolysate and the hydrolysate were centrifuged and filtered through gauzes, respectively, and then tile lipid layer in the supernatant was removed, The autolysate and the hydrolysate were finally concentrated under reduced atmospheric pressure in a rotary vacuum evaporator at $45^{\circ}C$ for 1 hour to produce the krill solubles in form of paste. The powdered krill solubles were prepared by the addition of $5\%$ starch to the autolysate and hydrolysate and by means of concentration in the rotary vacuum evaporator at $45^{\circ}C$ for 30 minutes and a forced air drying at $58^{\circ}C$ for 3 hours with a air velocity of 3m/sec. Among the amino acids in raw frozen krill, glutamic acid, lysine, and aspartic acid showed high values in quantity and then followed leucine, alanine, arginine, glycine and proline. The qnantity of histidine was very small and that of cystine was only in trace. The krill solubles in forms of paste and powder prepared by autolysis and hydrolysis with pronase-p revealed almost the same patterns in amino acid composition as in raw frozen krill. In case of free amino acids, a large quantity of it in raw frozen krill consisted of lysine, arginine, proline, alanine and leucine. The quantities of cystine, histidine and glutamic acid were, in contrast, very small. In the soluble krill paste prepared by autolysis, lysine, leucine, threonine and alanine existed in large quantities among the free amino acids and cystine, aspartic acid and histidine existed in small quantities. The contents of almost all of the free amino acids ill soluble krill paste perpared by hydrolysis with pronase-p were increased slightly as compared with those in soluble krill paste prepared by autolysis. In this product, the contents of cystine, histidine and serine were very low and lysine, leucine, arginine and proline were the dominant group in quantities among the free amino acids. The krill solubles in forms of paste and powder were not inferior to whole egg in the view point of its essential amino acid composition.

• Journal of the Korean Society of Clothing and Textiles
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• v.22 no.7
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• pp.843-850
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• 1998

The purpose of this study is the investigation of chemical properties of wool treated with oxidants and protease at low temperature. The chemical degradation of the fibers were investigated by measuring $\alpha$-amimo acid contents and FT-IR analysis. In addition, urea-hydrogensulfite solubility was measured to compare to the oxidation and protease treated wool. The results were as follows. 1) By the oxidation of wool, cystine is oxidised to cysteic acid by way of the intermediate oxides, cystine-S-monooxide and cystine-S-dioxide, in the case hydrolysis catalysed by the protease catalyse. Also, $\alpha$-amimo acid contents is increased, and urea-hydrogensulfite solubility was lower than that of untreated wool. This chemical degradation of wool was occurred due to oxidate hydrolysis in the order of permonosulfate>dichloroisocyanuric acid$\geq$chlorine. 2) The chemical degradation of wool was accelerated by the protease treatment of oxidized wool. Oxidation of wool is considered to make the fiber more susceptibled to enzymatic attact by opening disulphide bond within wool. Enzymatic attact was effectively directed to the wool oxidised by permonosulfate.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.42 no.4
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• pp.316-321
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• 2009

This study was conducted to improve the fish odor of extracts obtained from salmon frame. Salmon frame extracts were prepared using four kinds of pretreated salmon frame (salmon frame soaked in soybean milk and fried salmon frame) or containing additives (cystine and xylose-added salmon frame, and methionine and xylose-added salmon frame). Among the extracts prepared in this study, extracts containing cystine and xylose had the highest volatile component intensity and odor sensory score. These suggested that the fish odor of salmon frame extracts can be reduced by adding cystine and xylose before extraction.

• KSBB Journal
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• v.28 no.3
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• pp.191-195
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• 2013

Cordyceps militaris (CM) occurring from a fruiting body by a host insect is a kind of mushroom, which is composed of animal host and plant fruit body. CM contains large amounts of useful ingredients including polysaccharides, vitamins, amino acids, minerals, etc. The essential amino acids from CM including cystine, lysine, and methionine can be expected to improve damaged hair treatment as effective ingredients. In this study, the improvement effect of the CM extracts on chemical and physical properties for damaged-hair treatment was investigated. The cysteic acid and cystine monooxide produced by oxidation of cystine were analyzed their chemical structure by FT-IR spectroscopy. It was confirmed that the vibration absorption peak ($1,041cm^{-1}$) of cysteic acid was reduced and increased sulfur content considerably which means meaningful improvement effect on damaged-hair treatment. Apparently, the cuticle morphology of the damaged-hair was improved significantly by treatment with CM extracts. Especially, confocal laser scanning microscope images of the damaged-hair treated with the extract showed highly increased fluorescence intensity which means promising effect in hair treatment. The tensile strength of the damaged hair treated also increased by 168% compared with the damaged hair.

• Journal of the Korean Society of Clothing and Textiles
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• v.20 no.5
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• pp.728-737
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• 1996

The purpose of this study is to investigate the change of the chemical properties of wool treated with six kinds of alkali (NaOH, Naac03, NH40H, NH2CH3CH30H, TMAH and BTMAH) with or without CTAB. Content of bound fatty acid liberated from wool surface, elemental composition and allw6rden time were measured to compare the surface modification of untreated and alkali treated wool. Also, the chemical degradation of the fiber was investigated by measuring cystine contents and urea-hydrogensulfite solubility. The result were as follows: 1. By the alkali treatment of wool, the covalently bound fatty acid of the epicuticle was removed and the allworden time was shortened, and in the case of wool treated with TMAH, BTMAH, the allw6rden sacs were formed unevenly and rarely. Also, cystine contents and urea-hydrogensulfite solubility were decreased by alkali treatment on wool. 2. The modification of epicuticle and the chemical degradation of wool were occurred due to alkaline hydrolysis in the order of TMAH, BTMAH > NaOH, Na3c03> NH2CH3CHaOH, NH40H. 3. As a treating time increased, the modification of epicuticle and chemical degradation of wool were accelerated. By the addition of CTAB to the alkali solution, the modification of epicuticle was increase, and the cystine contents and urea-hydrogensulfite solubility were reduced than that of wool teated with alkli without CTAB due to reduction of negative charge on the wool surface by the adhesion of CTAB.