• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.1-9
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• 2016

Newborn screening (NBS) is important if early intervention is effective in a disorder and if there are sensitive and specific biochemical markers to detect disorder. Methionine is a useful marker to detect abnormal methionine-homocysteine metabolism, especially homocystinuria which needs urgent medical intervention. However, hypermethioninemia could occur in other metabolic disorder including liver disease, tyrosinemia type I, methionine adenosyltransferase (MAT) I/III deficiency, glycine N-methyltransferase (GNMT) deficiency, or adenosylhomocysteine hydrolase deficiency. However, experience with NBS for homocystinurias and methylation disorders is limited. Especially, MAT I/III deficiency which is the most common cause of persistent hypermethioninemia have two inheritance, autosomal recessive (AR) and autosomal dominant (AD), and their clinical manifestation is different between AR and AD. Here, author reviewed recent articles of guideline and proposed guideline for homocystinuria and methylation disorder.

• Food Science of Animal Resources
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• v.28 no.2
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• pp.138-145
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• 2008

This study was carried out to compare the meat quality of beef from Hanwoo supplemented with dietary Rhus verniciflua Stokes (RVS) meal, silicate (Si), and chromium-methionine (Cr-Met) during refrigerated storage. The 26 mon-aged Hanwoo steers were fed basal diets containing 4% RVS, 4% RVS+400 ppm Cr-Met, 1.4% $SiO_2$, or 0.14% $SiO_2+400 ppm$ Cr-Met for 4 mon prior to slaughter. The M. longissimus from carcasses were then stored at $4{\pm}0.2^{\circ}C$ for 7 d. The crude fat content was higher in the Si and Si+Cr-Met groups (p<0.05). The water-holding capacity (WHC) and tenderness were highest in the Si+Cr-Met group (p<0.05). With regard to fatty acids, the polyunsaturated fatty acid (PUFA) content was lower in the Si and Si+Cr-Met groups (p<0.05), and the monounsaturated fatty acid (MUFA) content was lowest in the Si+Cr-Met group (p<0.05). The TBARS and MetMb contents were decreased in the Si, Si+Cr-Met, and RVS+Cr-Met groups. Regarding meat color during storage, the Si+Cr-Met group showed the highest L, a, b, C values and total color, and those of the Si and RVS+Cr-Met groups were higher than the RVS group (p<0.05). Consequently, beef from Si-fed Hanwoo had higher fat content, color and oxidation stability, and lower PUFA content than RVS-fed beef. And beef from Si+Cr-Met-fed Hanwoo had higher WHC, tenderness and color stability, and lower MUFA content than Si-fed beef.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.329-334
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• 2010

Purpose : The neonatal screening test for homocystinuria primarily measures methionine by using a dried blood specimen. We investigated the incidence and clinical manifestations of homocystinuria, isolated hypermethioninemia, and transient hypermethioninemia among patients with hypermethioninemia on a neonatal screening test. Methods : We performed a retrospective study of 58 patients transferred to Shoonchunhyang Hospital because of hypermethioninemia on a neonatal screening test between January 1996 and August 2009. We analyzed the level of amino acid from plasma and urine, as well as blood homocysteine.Results : Almost half of the 58 patients were identified as normal. Whereas only 3 (5.1%) patients were identified as having homocystinuria, about 20.7% (12 cases) of the patients had isolated hypermethioninemia. The ages of these two groups at initial detection of hypermethioninemia on plasma amino acid analysis were $50.0{\pm}22.5$ days and $34.9{\pm}13.5$ days, respectively. Both groups were put on diets, and they showed a normal developmental course as a result of early diagnosis and treatment. Conclusion : Hypermethioninemia without homocystinuria, referred to as isolated hypermethioninemia, was also detected. Thus, the impact of hypermethioninemia on a neonatal screening test should be carefully evaluated through analysis of amino acid levels from blood and urine, and we need to detect and treat an early stage of isolated hypermethioninemia as well as homocystinuria.

• Monthly Duck's Village
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• s.114
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• pp.46-51
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• 2012

세계적으로 오리고기 생산량은 꾸준히 증가하고 있으며, 이에 따라 최적의 생산성을 이끌어 내기 위한 영양소 요구량 설정과 배합표 작성이 필요하다. 그러나 육지새와 물새의 해부학적, 생리학적, 대사적 차이점에도 불구하고 오리 연구 데이터가 희귀하다보니 오리사료 배합을 위해 육계의 자료를 참고하는 경우가 많다. 최근의 연구결과에 따르면 현재 오리 21일령까지 아미노산 권장수준은 라이신 1.16%, 메티오닌 0.76%, 메티오닌+시스틴 0.76%(Evonik, 2010)인데 이는 0~14일령 오리의 요구량에 가깝다. 15~35일령의 오리의 경우 증체를 위해서는 현 권장수준보다 다소 높은, 메티오닌 0.54%, 총 함황아미노산(TSAA) 0.84% 이상의 요구량을 필요로 할 것으로 보인다. 하지만 36~49일령 비육말기 오리 실험에서는 각각 0.35%, 0.59%로써 기존 추정량보다 낮은 것으로 나타났으며 35일령 이후에 보다 완만한 성장을 한 결과를 확인시켜주었다. 페킨종 오리의 생산성 향상을 위해서는 아미노산 요구량과 소화율에 대한 보다 심도 있고 추가적인 연구가 필요할 것이다.

• KOREAN POULTRY JOURNAL
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• v.24 no.2 s.268
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• pp.155-159
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• 1992

• Korean Journal of Food Science and Technology
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• v.12 no.4
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• pp.263-271
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• 1980

Detoxified and deallergenized castor bean protein isolate was prepared from defatted castor bean pomace for use in animal feedstuffs and human foods. Succinylation and acetylation of the ${\varepsilon}-amino$ groups of the protein improved markedly the water solubility of the protein at $pH\;7{\sim}8$. The results of the amino acid analysis of the protein isolate revealed that the sulfur-containing amino acids and L-lysine were limiting amino acids and that succinylation and acetylation caused some little loss of the amino acid content. The L-methionine enriched plastein was synthesized from the protein isolate or the acylated protein isolates and DL-methionine ethyl ester by one step process with papain. By this method the extent of incorporation of L-methionine was about 50%. Pepsin hydrolyzed both unmodified and modified protein isolates at the same rate (about 92%). Tryptic hydrolysis, however, was less for the succinylated protein isolates (about 42%) and less for the acetylated protein isolates (about 26%). The protein efficiency ratio of L-methionine enriched protein isolate (about 2.5 weight %) was 90% that of reference casein. The protein efficiency ratio values of succinylated (88%) and acetylated (84%) protein isolate were 55 and 69% of reference casein, respectively.

• Journal of the Korean Chemical Society
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• v.54 no.6
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• pp.680-686
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• 2010

Inhibition effects of alanine(Ala), asparagine(Asn), aspartic acid(Asp), glutamine(Gln) and methionine(Met) on the corrosion of copper were investigated in aerated artificial sea water. Amino acid adsorption process in copper surface can be explained by Temkin logarithmic isotherm due to the interaction between the adsorbed molecules. The inhibition efficiency for the copper corrosion depended on the concentration of amino acids.

• Journal of Plant Biology
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• v.34 no.1
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• pp.31-35
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• 1991

Induction of symbiosis between N. muscorum and cultured tobacco cells associately cultured on nitrogen-free medium and effects of polyamines on culture condition were carried out. Analysis of amino acid composition in associate cultures showed increase in total amino acid amounts than single culture of cultured tobacco cells and methionine was markly increased in associative culture on N-free medium treated with 1 mM spermine. These results indicated that the composition of amino acids increased effectively in associative cultures by nitrogen fixation of N. muscorum.scorum.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.11 no.1
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• pp.103-105
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• 2011

고호모시스틴혈증(hyperhomocysteinemia)은 호모시스테인과 메티오닌의 대사과정에 관여하는 여러 효소들의 결핍에 의해 발생할 수 있으며, 대표적인 효소 결핍으로는 cysthathione beta-synthase (CBS) 결핍증, Methionine synthase (MS) 결핍증, methylenetetrahydrofolate reductase (MTHFR) 결핍증이 있다. 이들은 고호모시스테인혈증을 보이나 임상증상, 메티오닌의 동반 상승, 거대적아구성빈혈, 메칠말로닌산뇨증등의 동반 여부등을 토대로 감별진단에 도움을 받을 수 있다. 본 연자는 뇌혈전증과 뇌졸중을 동반하는 고호모시스테인혈증의 원인으로 MTHFR 유전자의 돌연변이를 발견한 증례를 경험하였기에 이를 보고하는 바이다.

• Journal of the Korean Chemical Society
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• v.59 no.2
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• pp.125-131
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• 2015

Inhibition effects of histidine (His), methionine (Met) on the corrosion of nickel were investigated in deaerated 0.5 M HCl and 0.5 M $H_2SO_4$ solution. All the inhibition efficiency for the nickel corrosion depended on the anodic inhibition. Amino acid adsorption process on nickel surface in the solution of HCl can be explained by modified Langmuir isotherm, however, in the solution of $H_2SO_4$ by Temkin logarithmic isotherm due to the interaction between the adsorbed molecules. The molecule of histidine dissolved in HCl-solution were physically adsorbed due to the electrostatic interaction between the surface of {$Ni-Cl^-$} and the {$-NH{_3}^+$} and {$-NH^+=$} of His. However the other cases of adsorption in this investigation can be explained by chemical adsorption between the empty d-orbital of Ni and the lone pair of electron in His and Met.