Literature DB >> 8487492

Identification of N-acetyl-alpha-aminoadipic acid in the urine of a patient with alpha-aminoadipic and alpha-ketoadipic aciduria.

T Takechi1, T Okada, H Wakiguchi, H Morita, T Kurashige, K Sugahara, H Kodama.   

Abstract

A 3.5-year-old Japanese boy with a mild speech disturbance excreted a large amount of alpha-aminoadipic acid into the urine. The amino acid analysis using an amino acid analyser confirmed the presence of alpha-aminoadipic acid in both urine and plasma. We detected alpha-aminoadipic acid in the hydrolysate of the effluent and washwater fraction through cation exchange resin. This suggested the presence of acetylated derivatives and we identified N-acetyl-alpha-aminoadipic acid using liquid chromatography-atmospheric pressure ionization mass spectrometry (LC-API-MS). The concentrations of alpha-aminoadipic acid and N-acetyl-alpha-aminoadipic acid in the urine of a patient with alpha-aminoadipic aciduria were 376.9 nmol/mg creatinine and 18.1 nmol/mg creatinine, respectively. We also detected alpha-ketoadipic acid in the urine of this patient using LC-API-MS.

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Year:  1993        PMID： 8487492     DOI： 10.1007/bf00711325

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  14 in total

1.  N-acetylaspartic aciduria in Canavan disease: another proof in two infants.

Authors:  K Yalaz; M Topçu; H Topaloğlu; O Gürçay; O E Ozcan; B Onol; Y Renda
Journal:  Neuropediatrics       Date:  1990-08       Impact factor: 1.947

2.  Alpha-ketoadipic aciduria: a description of a new metabolic error in lysine-tryptophan degradation.

Authors:  R W Wilson; C M Wilson; S C Gates; J V Higgins
Journal:  Pediatr Res       Date:  1975-06       Impact factor: 3.756

3.  Alpha-aminoadipic aciduria, a non-deleterious inborn metabolic defect.

Authors:  M H Fischer; T Gerritsen; J M Opitz
Journal:  Humangenetik       Date:  1974

4.  A patient with alpha-ketoadipic and alpha-aminoadipic aciduria.

Authors:  M Duran; F A Beemer; S K Wadman; U Wendel; B Janssen
Journal:  J Inherit Metab Dis       Date:  1984       Impact factor: 4.982

5.  Biochemical and clinical studies of a new case of alpha-aminoadipic aciduria.

Authors:  R E Casey; W A Zaleski; M Philp; I S Mendelson; S L MacKenzie
Journal:  J Inherit Metab Dis       Date:  1978       Impact factor: 4.982

6.  Alpha-ketoadipic aciduria: degradation studies with fibroblasts,.

Authors:  U Wendel; H W Rüdiger; H Przyrembel; H J Bremer
Journal:  Clin Chim Acta       Date:  1975-02-08       Impact factor: 3.786

7.  alpha-Aminoadipic aciduria and persistence of fetal haemoglobin in an oligophrenic child.

Authors:  A J Manders; C G von Oostrom; J M Trijbels; F J Rutten; W J Kleijer
Journal:  Eur J Pediatr       Date:  1981-03       Impact factor: 3.183

8.  Alpha-aminoadipic aciduria: chemical and enzymatic studies.

Authors:  R G Gray; E M O'Neill; R J Pollitt
Journal:  J Inherit Metab Dis       Date:  1980       Impact factor: 4.982

9.  Metabolism of alpha-aminoadipic and alpha-ketoadipic acids: studies using rat and beef liver, and human leukocytes.

Authors:  R W Wilson; C M Wilson; J V Higgins
Journal:  Clin Chim Acta       Date:  1976-06-01       Impact factor: 3.786

10.  Tryptophan and lysine metabolism in alpha-aminoadipic aciduria.

Authors:  M H Fischer; R R Brown
Journal:  Am J Med Genet       Date:  1980
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  6 in total

1.  A study on alpha-ketoadipic aciduria by gas chromatographic-mass spectrometry.

Authors:  Zhen-Wei Xia; Yoshito Inoue; Morimasa Ohse; Toshihiro Shinka; Tomiko Kuhara
Journal:  World J Gastroenterol       Date:  2000-10       Impact factor: 5.742

2.  α-Ketoadipic Acid and α-Aminoadipic Acid Cause Disturbance of Glutamatergic Neurotransmission and Induction of Oxidative Stress In Vitro in Brain of Adolescent Rats.

Authors:  Janaína Camacho da Silva; Alexandre Umpierrez Amaral; Cristiane Cecatto; Alessandro Wajner; Kálita Dos Santos Godoy; Rafael Teixeira Ribeiro; Aline de Mello Gonçalves; Ângela Zanatta; Mateus Struecker da Rosa; Samanta Oliveira Loureiro; Carmen Regla Vargas; Guilhian Leipnitz; Diogo Onofre Gomes de Souza; Moacir Wajner
Journal:  Neurotox Res       Date:  2017-04-20       Impact factor: 3.911

3.  Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria.

Authors:  Jacob Hagen; Heleen te Brinke; Ronald J A Wanders; Alida C Knegt; Esmee Oussoren; A Jeannette M Hoogeboom; George J G Ruijter; Daniel Becker; Karl Otfried Schwab; Ingo Franke; Marinus Duran; Hans R Waterham; Jörn Oliver Sass; Sander M Houten
Journal:  J Inherit Metab Dis       Date:  2015-04-10       Impact factor: 4.982

4.  Substrate specificity and structure of human aminoadipate aminotransferase/kynurenine aminotransferase II.

Authors:  Qian Han; Tao Cai; Danilo A Tagle; Howard Robinson; Jianyong Li
Journal:  Biosci Rep       Date:  2008-08       Impact factor: 3.840

5.  Regulation of oxidative degradation of L-lysine in rat liver mitochondria.

Authors:  P W Scislowski; A R Foster; M F Fuller
Journal:  Biochem J       Date:  1994-06-15       Impact factor: 3.857

6.  Functional Characterization of CYP94-Genes and Identification of a Novel Jasmonate Catabolite in Flowers.

Authors:  Viktoria Bruckhoff; Sven Haroth; Kirstin Feussner; Stefanie König; Florian Brodhun; Ivo Feussner
Journal:  PLoS One       Date:  2016-07-26       Impact factor: 3.240
  6 in total

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