Literature DB >> 2466169

Homocitrullinuria and homoargininuria in hyperargininaemia.

T Kato1, M Sano, N Mizutani, C Hayakawa.   

Abstract

A four-year-old boy with hyperargininaemia had increased urinary excretion of homocitrulline and homoarginine. A single oral lysine load created a marked increase in these amino acids in plasma. A daily oral lysine supplementation resulted in a remarkable urinary leakage of homocitrulline and homoarginine. These findings suggest that the patient had an enhanced synthesis of these amino acids.

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Year:  1988        PMID： 2466169     DOI： 10.1007/bf01800367

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


  10 in total

Review 1.  Enzymes of arginine and urea synthesis.

Authors:  S Ratner
Journal:  Adv Enzymol Relat Areas Mol Biol       Date:  1973

2.  Citrullinemia: a new case, with implications concerning adaptation to defective urea synthesis.

Authors:  A Scott-Emuakpor; J V Higgins; A F Kohrman
Journal:  Pediatr Res       Date:  1972-07       Impact factor: 3.756

3.  Saccharopinuria: a new inborn error of lysine metabolism.

Authors:  N A Carson; B G Scally; D W Neill; L J Carré
Journal:  Nature       Date:  1968-05-18       Impact factor: 49.962

4.  Lysine, homocitrulline, and homoarginine metabolism by the isolated perfused rat liver.

Authors:  W L Ryan; A J Barak; R J Johnson
Journal:  Arch Biochem Biophys       Date:  1968-02       Impact factor: 4.013

5.  Homoarginine synthesis by rat kidney.

Authors:  W L Ryan; R J Johnson; S Dimari
Journal:  Arch Biochem Biophys       Date:  1969-05       Impact factor: 4.013

6.  Homoargininosuccinic acid synthesis by an enzyme from pig kidney.

Authors:  J J Strandholm; N R Buist; N G Kennaway
Journal:  Biochim Biophys Acta       Date:  1971-05-18

7.  Hyperargininemia.

Authors:  S D Cederbaum; K N Shaw; M Valente
Journal:  J Pediatr       Date:  1977-04       Impact factor: 4.406

8.  Guanidino compounds in plasma, urine and cerebrospinal fluid of hyperargininemic patients during therapy.

Authors:  B Marescau; I A Qureshi; P De Deyn; J Letarte; R Ryba; A Lowenthal
Journal:  Clin Chim Acta       Date:  1985-02-28       Impact factor: 3.786

9.  Lysine intolerance in a variant form of citrullinemia.

Authors:  I Matsuda; S Arashima; Y Imanishi; J Yamamoto; I Akaboshi; S Shinozuka; N Nagata
Journal:  Pediatr Res       Date:  1979-10       Impact factor: 3.756

10.  Increased urinary excretion of putrescine in hyperargininaemia.

Authors:  T Kato; M Sano; N Mizutani; C Hayakawa
Journal:  J Inherit Metab Dis       Date:  1987       Impact factor: 4.982
  10 in total
  1 in total

1.  Homocitrullinuria and homoargininuria in lysinuric protein intolerance.

Authors:  T Kato; M Sano; N Mizutani
Journal:  J Inherit Metab Dis       Date:  1989       Impact factor: 4.982
  1 in total

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