- Homo-Genius: Homocitrulline Can Be a Better Target than Citrulline as a Biomarker for Rheumatoid Arthritis?
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Shim SC
- KMID: 2371671
- J Rheum Dis.
- 2017 Feb;24(1):1-3.
- doi: 10.4078/jrd.2017.24.1.1
No abstract available. -
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- A Case of Citrullinemia
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Park DS, Kim DU, Moon SO, Lee IJ
- KMID: 2142438
- J Korean Pediatr Soc.
- 1997 Apr;40(4):584-587.
Citrullinemia is one of the five urea cycle defects and is caused by argininosuccinic acid synthetase deficiency ; conversion of citrulline to argininosuccinic acid is blocked. Severe hyperammonemia typically develops... -
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- Assessment of Endogenous Nitric Oxide Formation in the Neonate : Measurement of Urinary Nitrite and Citrulline Concentrations
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Kim YH, Yu KH, Hong YS, Kim SK
- KMID: 1605934
- J Korean Pediatr Soc.
- 2001 Jan;44(1):40-45.
PURPOSE: At birth, as the fetus adjusts to the "new world", the fetal pulmonary circulation undergoes a striking transition, characterized by an immediate rise in pulmonary blood flow and a... -
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- A Case of Citrullinemia Controlled by Diet and Arginine
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Jeong EY, Jeon SS, Seo SS
- KMID: 2099009
- J Korean Soc Neonatol.
- 1999 Nov;6(2):280-280.
Citrullinemia is an inborn error of urea cycle metabolism caused by deficiency of arginosuccinate synthetase. It is characterized by hyperammonemia and high citrulline level in serum, CSF and urine. The... -
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- Novel Compound Heterozygote Mutations of the SLC25A13 Gene in an Infant with Neonatal-onset Type II Citrullinemia Detected by Newborn Mass Screening
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Lim HS, Kim H, Kim SS, Kim GH, Yoo HW, Shin YL
- KMID: 2099215
- J Korean Soc Neonatol.
- 2011 Nov;18(2):370-373.
- doi: 10.5385/jksn.2011.18.2.370
Citrin deficiency caused by the SLC25A13 gene mutations is associated with both neonatal-onset type II citrullinemia (CTLN2), also known as neonatal intrahepatic cholestasis caused by citrin deficiency and adult-onset CTLN2.... -
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- Peptidylarginine Deiminase and Citrullination: Potential Therapeutic Targets for Inflammatory Diseases
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Jang B, Shin SJ
- KMID: 2286269
- J Bacteriol Virol.
- 2013 Sep;43(3):159-167.
- doi: 10.4167/jbv.2013.43.3.159
The multiple post-translational modifications of proteins display specific gain- or loss-of-function under normal and abnormal conditions. These modifications are precisely regulated by post-translational modification enzymes. The altered molecular status perturbs... -
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- A Case of Ornithine Transcarbamylase (OTC) Deficiency
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Kwon S, Lee Y, Choe BH, Lee S
- KMID: 1946283
- J Korean Pediatr Soc.
- 2000 Jan;43(1):123-127.
OTC deficiency is an X-linked disorder in which the synthesis of urea is impaired. OTC catalyzes the synthesis of citrulline from carbamyl phosphate and ornithine. Complete or partial deficiencies of... -
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- A Novel Argininosuccinate Synthetase Gene Mutation in a Korean Family with Type I Citrullinemia
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Ahn BW, Kim HJ, Park HD, Kim WD
- KMID: 2321496
- J Korean Soc Neonatol.
- 2010 Nov;17(2):250-253.
Citrullinemia type I is an urea cycle defect caused by mutations in the argininosuccinate synthetase (ASS1) gene. We report a novel argininosuccinate synthetase gene mutation in a Korean family with... -
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- A Case of Adult-type Citrullinemia with Hyperammonemia
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Park HJ, Lim HJ, Jung IS, Kim YH, Kim IH, Chung IK, Kim HS, Park SH, Lee MH, Kim SJ, Lee DH
- KMID: 2085022
- Korean J Gastroenterol.
- 2002 May;39(5):379-385.
Adult type citrullinemia is a urea cycle enzymopathy, which is characterized by hyperammonemia, high citrulline level in serum and decreased activity of argininosuccinic acid synthetase in liver biopsy. Clinical symptoms... -
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- Successful treatment of a child with citrullinemia
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Lee KH, Park MS, Hahn SH
- KMID: 1963057
- J Genet Med.
- 1997 Sep;1(1):5-10.
The amino acids formed by degradation of proteins ingested produce ammonia. The ammonia which is broken down and excreted as urea through a process known as the Klebs-Hensleit cycle or... -
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- The Effects of Repeated Toluene Exposure on Amino Acid Neurotransmitters in the Rat Brain
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Kim HK, Baeck SK, Ryu SJ, Kim IS
- KMID: 2228770
- Korean J Crit Care Med.
- 1998 Jun;13(1):33-42.
Introduction: It was aimed to investigate the effect of chronic toluene exposure on amino acid neurotransmitters in the rat brain, corpus striatum. METHODS: Twenty four male Sprague-Dawley rats were divided... -
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- Anesthetic care for living donor auxiliary partial orthotopic liver transplantation in the treatment of adult-onset type II citrullinemia: A case report
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Paek CM, Chung JY, Yi JW, Lee BJ, Kim DO, Kang JM
- KMID: 2299213
- Korean J Anesthesiol.
- 2008 Aug;55(2):244-249.
- doi: 10.4097/kjae.2008.55.2.244
A deficiency of the urea cycle enzyme, argininosuccinate synthetase which is produced in liver, makes citrullinemia, which is an autosomal recessive disorder. As the liver is the only organ which... -
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- Serum Amino Acid Levels in Term and Preterm Neonates
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Youn YS, Kim SZ, Chang MY
- KMID: 2188581
- J Korean Soc Neonatol.
- 2006 May;13(1):90-96.
PURPOSE: This study was aimed to analyze the level of serum amino acids according to the sex, birth weight, gestational age in neonates. METHODS: Amino acid was measured by tandem... -
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- Anesthesia for Living Related Liver Transplantation in Classic Citrullinemia: A case report
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Huh IY, Hwang KS, Park CH, Park SW, Shin JW, Chio KT
- KMID: 2237546
- Korean J Anesthesiol.
- 2004 Aug;47(2):287-291.
- doi: 10.4097/kjae.2004.47.2.287
Citrullinemia is an autosomal recessive disorder resulting in a deficiency of the urea cycle enzyme, argininosuccinate synthetase, which is mainly found in the liver. Despite the improvement in a dietary... -
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- A Case of Citrullinemia Diagnosed at the Neonatal Period
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Song SK, Oh KC, Hong MA, Kim HT, Shin HJ, Kim SY, Chang JK, Jo HS, Kim BI, Yang SW, Choi JH
- KMID: 2335624
- J Korean Pediatr Soc.
- 2002 Apr;45(4):524-528.
Citrullinemia is a rare inborn error of metabolism of the urea cycle, and was first reported by McMurray, et al. in 1962. It is inherited as an autosomal recessive trait.... -
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- A Case of Molecular Diagnosis of Ornithine Transcarbamylase Deficiency
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Lee ES
- KMID: 2320145
- Yeungnam Univ J Med.
- 2007 Dec;24(2):322-328.
Ornithine transcarbamylase (OTC) deficiency is the most common inborn error of urea cycle metabolism; it is inherited in an X-linked manner. The OTC catalyzes the third step of the urea... -
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- Auxiliary partial orthotopic liver transplantation for adult onset type II citrullinemia
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Kim BS, Joo SH, Lee SH, Lee JI, Kim HC, Nam DH, Park HC
- KMID: 2096651
- J Korean Surg Soc.
- 2011 Jun;80(Suppl 1):S51-S54.
- doi: 10.4174/jkss.2011.80.Suppl1.S51
Adult-onset type II citrullinemia (CTLN2) is a disorder caused by an inborn error of metabolism affecting the liver. CTLN2 is an autosomal recessive disorder characterized by recurrent encephalopathy with hyperammonemia... -
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- Histochemical Localization of NADPH-diaphorase in Olfactory System of Rat
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Lee HM, Choi CS, Lee SH
- KMID: 1922549
- Korean J Otolaryngol-Head Neck Surg.
- 1997 Apr;40(4):595-599.
Nitric oxide(NO) has recently been identified as a short-lived intracellular messenger in the central and peripheral nervous systems. Nitric oxide synthase(NOS) is a key enzyme for NO biosynthesis by formation... -
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- A Case of Ornithine Transcarbamylase Deficiency Successfully Treated with Protein Restriction and Living Related Liver Transplantation
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Kim BS, Kim KM, Yoo HW, Lee SG
- KMID: 2041992
- J Korean Pediatr Soc.
- 1999 Jun;42(6):868-873.
Ornithine transcarbamylase deficiency(OTCD), the most common inborn error of the urea cycle, is inherited in X-linked manner. In affected hemizygote males, OTCD manifests hyperammonemic coma that often leads to death... -
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- Short Bowel Syndrome as the Leading Cause of Intestinal Failure in Early Life: Some Insights into the Management
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Goulet O, Abi Nader , Pigneur B, Lambe C
- KMID: 2451572
- Pediatr Gastroenterol Hepatol Nutr.
- 2019 Jul;22(4):303-329.
- doi: 10.5223/pghn.2019.22.4.303
Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe... -
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