Literature DB >> 6796766

Alpha-aminoadipic aciduria: chemical and enzymatic studies.

R G Gray, E M O'Neill, R J Pollitt.   

Abstract

A new case of alpha-aminoadipic aciduria had an apparent immunodeficiency and died at the age of 4 months. The urine contained large amounts of alpha-aminoadipate and smaller quantities of alpha-keto- and alpha-hydroxyadipate. Post mortem, the highest concentrations of alpha-aminoadipate were found in liver and kidney. Enzymatic studies on liver and cultured fibroblasts failed to demonstrate the expected deficiency of alpha-amino-adipate aminotransferase, a result perhaps explicable by the presence of cytoplasmic aminotransferase activity.

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Year:  1980        PMID： 6796766     DOI： 10.1007/BF01805664

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


  12 in total

1.  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

2.  Alpha-Aminoadipate aminotransferase and kynurenine aminotransferase. Purification, characterization, and further evidence for identity.

Authors:  M C Tobes; M Mason
Journal:  J Biol Chem       Date:  1977-07-10       Impact factor: 5.157

3.  Subcellular localization and tissue distribution of alpha-ketoadipate reduction and oxidation in the rat.

Authors:  T Suda; J C Robinson; T A Fjellstedt
Journal:  Biochem Biophys Res Commun       Date:  1977-07-25       Impact factor: 3.575

4.  Alpha-amino adipic aciduria in an oligophrenic child.

Authors:  S Lormans; A Lowenthal
Journal:  Clin Chim Acta       Date:  1974-11-20       Impact factor: 3.786

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

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

6.  Alpha-aminoadipate aminotransferase of rat liver mitochondria.

Authors:  Y Nakatani; M Fujioka; K Higashino
Journal:  Biochim Biophys Acta       Date:  1970-02-11

7.  Alpha-ketoadipic aciduria, a new inborn error of lysine metabolism; biochemical studies.

Authors:  H Przyrembel; D Bachmann; I Lombeck; K Becker; U Wendel; S K Wadman; H J Bremer
Journal:  Clin Chim Acta       Date:  1975-02-08       Impact factor: 3.786

8.  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

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.  Glutaric aciduria: clinical and laboratory findings in two brothers.

Authors:  N Gregersen; N J Brandt; E Christensen; I Gron; K Rasmussen; S Brandt
Journal:  J Pediatr       Date:  1977-05       Impact factor: 4.406
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  7 in total

1.  alpha-Aminoadipic and alpha-ketoadipic aciduria: detection of a new case by a screening program using two-dimensional thin layer chromatography of amino acids.

Authors:  C Vianey-Liaud; P Divry; J Cotte; G Teyssier
Journal:  J Inherit Metab Dis       Date:  1985       Impact factor: 4.982

2.  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

3.  Sudden death of a 2-year-old child due to alpha-ketoadipic aciduria.

Authors:  Hiroki Kondou; Hiroaki Ichioka; Yoshihisa Akasaka; Hidehito Kondo; Hiroshi Ikegaya
Journal:  Forensic Sci Med Pathol       Date:  2022-06-01       Impact factor: 2.007

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

Authors:  T Takechi; T Okada; H Wakiguchi; H Morita; T Kurashige; K Sugahara; H Kodama
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

5.  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

6.  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

7.  Abnormal alpha-aminoadipic acid excretion in a newborn with a defect in platelet aggregation and antenatal cerebral haemorrhage.

Authors:  M Candito; C Richelme; P Parvy; C Dageville; A Appert; S Bekri; D Rabier; P Chambon; R Mariani; P Kamoun
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982
  7 in total

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