• Childhood Kidney Diseases
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• 제27권2호
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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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• 제17권1호
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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.

• 한국식품영양과학회지
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• 제11권3호
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• pp.37-40
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• 1982

한국산(韓國産) 홍삼(紅蔘), 한국(韓國), 미국(美國) 및 카나다산(産) 피부백삼(皮部白蔘)으로부터 tryptophan, proline, cystine을 제외한 15 종의 유리 아미노산을 고속액체(高速液體) 크로마토그래피로 검출해 낼 수 있었다. 이들 아미노산 중에서 arginine이 홍삼(紅蔘)에서는 총 유리 아미노산의 72.6%를, 피부백삼류(皮部白蔘類)에서는 $48.2{\sim}68.7%$나 차지하고 있었다. 한국산(韓國産) 홍삼(紅蔘)의 각 유리 아미노산 함량은 한국산(韓國産) 피부백삼(皮部白蔘)에 비하여 많이 감소되어 있는 경향이었고, 미국산(美國産) 및 카나다산(産) 피부백삼(皮部白蔘)의 경우 한국산(韓國産) 그것에 비하여 대체로 각 유리 아미노산 함량이 낮은 경향을 보여 주었다. 한국산(韓國産) 홍삼(紅蔘) 추출물(抽出物)에서는 tryptophan, proline, cystine 이외에도 methionine, phenylalanine등이 검출되지 않았다. 이들 추출물(抽出物)에서도 역시 arginine이 가장 많이 함유되어 있었고 각 유리 아미노산 함량은 백삼추출물(白蔘抽出物)에서 더 많았다.

• Applied Biological Chemistry
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• 제9권
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• pp.59-64
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• 1968

1. 국자(?子)를 사용(使用)하여 백미(白米) 및 옥수수를 원료(原料)로 탁주(濁酒)를 담금하고 경시적(經時的)으로 그 발효(醱酵)술덧중(中)의 Amino-N를 Formol 법(法)으로 정량(定量)하고 또한 유리(遊離) Amino 산(酸)을 PPC 법(法)으로 검출(檢出).하였다. 2. 백미정료탁주(白米定料濁酒)술 덧 중(中)에서는 Aspartic acid, Cystine Glutamic acid, Glycine, Serine Alanine, Tyrosine, Histidine, Valine, Tryptophan, Phenylalanine, Proline 및 Leucine 등 14종의 Amino 산(酸)을 검출(檢出)하였다. 3. 옥수수원료탁주(原料濁酒)술덧 중(中)에서는 Aspartic acid, Cystine, Glycine, Derine, Alanine, Lysine, Valine, Aistididine, Proline, Leucine 및 Tryptophan 등(等) 12종(種의) Amino 산(酸)을 검출하였다. 4. 백미(白米) 및 옥수수원료(原料) 탁주중(濁酒中)의 주체 Amino 산(酸)은 Paper Chromatography에 나타난 각(各) Spot의 정색도(呈色度)로 보아 Lysine, Valine, Leucine, Serine, Proline 및 Glycine 등(等)으로 추정(推定)하였다.

• 대한유전성대사질환학회지
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• 제4권1호
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• pp.23-29
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• 2004

시스틴증은 상염색체 열성으로 발생하는 매우 드문 용해소체 축적병으로서, 용해소체 막을 통한 시스틴 운반의 장애로 발생한다. 용해소체 내 시스틴의 축척과 결정화에 의해 전신의 여러 조직에 파괴가 진행되어 성장 장애, Fanconi 증후군, 신부전, 눈의 이상, 내분비 질환 등이 나타난다. 치료제인 cysteamine의 투여가 예후를 향상시킬 수 있으므로 조기 진단과 치료가 중요하다. 저자들은 성장 장애를 주소로 내원한 12세 남아에서 slit-lamp 검사와 혈액 백혈구 시스틴 농도 측정을 통하여 시스틴증을 확진하고 cysteamine 치료를 시행하면서 추적 관찰 중이다.

• 한국수산과학회지
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• 제12권4호
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• pp.235-240
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• 1979

krill을 식용화하기 위한 한 방법으로서 paste상 및 분말상의 krill soluble을 가공하고 제품의 구성아미노산 및 유리아미노 리의 조성을 검토하였다. 생동결 krill, 자가소화 및 $0.2\%$의 pronase-p의 첨가후의 가수분해에 의한 paste상과 분말상의 krill solube의 구성아미노산 중에는 glutamic acid, lysinge 및 aspartic acid가 가장 많았으며, cystine과 histidine이 가장 적었다. 생동결 krill의 유리아미노산중에는 lysine, arginine, proline, glycine, alanine 및 leucine이 가장 많았으며, cystine과 histidine 그리고 구성아미노산에서 그 함량이 많던 glutamic acid와 aspartic acid가 가장 적었다. 자가소화에 의한 paste상의 krill soluble의 유리아미노산에서는 생동결 krill에서 보다 glutamin acid, serine threonine, isoleucine, aspartic acid, valine 등의 량이 많아졌으며, 특히 glutamic acid는 거의 40배정도로 증가하였다. 전체적으로는 lysine, leucine, threonine 및 alanine 등이 많았으며 cystine, aspartic acid 및 histidine의 량이 적었다. $0.2\%$의 pronase-p를 첨가하여 가수분해한 paste상의 krill soluble 유리아미노산에는 aspartic acid와 glutamic acid이 량이 자가소화시보다 월등히 큰 증가폭을 보였으며 전체적으로는 lysine, leucine, arginine, alanine 및 proline이 많았으며 cystine, histidine, serine 등의 량이 적었다. 생동결 krill 및 drill soluble의 필수아미노산은 함량면에서 달걀과 비교하여 손색이 없었다.

• 한국의류학회지
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• 제22권7호
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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.

• 한국수산과학회지
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• 제42권4호
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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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• 제28권3호
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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.

• 한국의류학회지
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• 제20권5호
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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.