- A Case of Cystathioninuria with Delayed Development
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Byun SH, Lee HS, Pee DH, Lee KH, Eun BL, Hahn SH
- KMID: 2329309
- J Korean Child Neurol Soc.
- 2003 May;11(1):184-190.
Cystathionine is well-known intermediate in the metabolism of methionine. It is cleaved to cysteine and homoserine by gamma-cystathionase. This enzyme utilize pyridoxal 5'-phosphate as coenzyme. gamma-cystathionase deficiency leads to persistent... -
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- A Case of Homocystinuria with Ectopia Lentis
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Chung DY, Chung YT
- KMID: 2122983
- J Korean Ophthalmol Soc.
- 1991 Jan;32(1):110-115.
Homocystinuria is an inborn error on the pathway of the methionine metabolism. It is mainly caused by a cystathionine B-synthase deficiency in the brain or liver. Homocystinuria is biochemically characterized... -
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- Volatile Sulfur Compounds as a Predictor for Esophagogastroduodenal Mucosal Injury
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Yoo SH, Jung HS, Sohn WS, Kim BH, Ku BH, Kim YS, Park SW, Hahm KB
- KMID: 2135129
- Gut Liver.
- 2008 Sep;2(2):113-118.
BACKGROUND/AIMS: Halitosis is a symptom that bothers patients more socially than medically and its pathogenic mechanisms are unclear and treatment armamenterium is limited. Clinicians generally ignored active interventions. Since halitosis... -
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- An Adolescent Ischemic Stroke Patient with Hyperhomocysteinemia, MTHFR 677TT and CBS 1080TT genotypes
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Lee DH, Park CI, Lee IH, Lee JM, Kim SH, Lee JY, Lee SS, Choi BO
- KMID: 1626913
- J Korean Neurol Assoc.
- 2005 Jun;23(3):382-385.
Hyperhomocysteinemia is an independent risk factor for cerebrovascular disease. Hyperhomocysteinemia can be caused by the defect of the remethylation pathway including the 5, 10-methylenetetrahydrofolate reductase (MTHFR) gene or the transsulfuration... -
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- Beneficial Role of Hydrogen Sulfide in Renal Ischemia Reperfusion Injury in Rats
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Choi EK, Park SH, Lim JA, Hong SW, Kwak KH, Park SS, Lim DG, Jung H
- KMID: 2419730
- Yonsei Med J.
- 2018 Oct;59(8):960-967.
- doi: 10.3349/ymj.2018.59.8.960
PURPOSE: Hydrogen sulfide (H2S) is an endogenous gaseous molecule with important physiological roles. It is synthesized from cysteine by cystathionine γ-lyase (CGL) and cystathionine β-synthase (CBS). The present study examined... -
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- Age-Related Changes in Sulfur Amino Acid Metabolism in Male C57BL/6 Mice
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Jeon JS, Oh JJ, Kwak HC, Yun HY, Kim HC, Kim YM, Oh SJ, Kim SK
- KMID: 2429056
- Biomol Ther.
- 2018 Mar;26(2):167-174.
- doi: 10.4062/biomolther.2017.054
Alterations in sulfur amino acid metabolism are associated with an increased risk of a number of common late-life diseases, which raises the possibility that metabolism of sulfur amino acids may... -
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- Recombinant adeno-associated virus mediated gene transfer in a mouse model for homocystinuria
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Park ES, Oh HJ, Kruger WD, Jung SC, Lee JS
- KMID: 1115943
- Exp Mol Med.
- 2006 Dec;38(6):652-661.
Homocystinuria is a metabolic disorder caused by a deficiency of cystathionine b-synthase (CBS). The major clinical symptoms of this disease are mental retardation, lens dislocation, vascular disease with life-threatening thromboembolisms,... -
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- Plasma Homocysteine Concentration and Genotype Variation of Enzymes as Risk Factors in Patients with Coronary Artery Disease
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Kim SG, Kim YD, Kim SG, Jang SJ, Choi HJ, Kim BK, Lee SH, Park TH, Yang DG, Cha KS, Kim MH, Kim JS, Han JY, Kim JM
- KMID: 2029562
- Korean Circ J.
- 2001 Aug;31(8):757-766.
- doi: 10.4070/kcj.2001.31.8.757
BACKGROUND AND OBJECTIVES: Increased plasma homocysteine(tHcy) has been implicated as an independent risk factor for coronary artery diseas(CAD), but the relationship has not been firmly established. Present study aimed to... -
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