Literature DB >> 6434824

Threonine dehydratase deficiency: a probable cause of non-ketotic hyperglycinaemia.

I Krieger, F Booth.   

Abstract

A patient with classical symptoms of non-ketotic hyperglycinaemia (NKH) is presented. Threonine dehydratase was undetectable in a liver autopsy specimen, which was obtained within 1 h of death and immediately frozen at -70 degrees C. Activities of four marker enzymes were normal. This represents the first documentation of an inborn error of threonine metabolism and a new explanation of NKH.

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Year:  1984        PMID： 6434824     DOI： 10.1007/bf01805800

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


  15 in total

1.  Studies on the nature, inducibility, and assay of the threonine and serine dehydrase activities of rat liver.

Authors:  L GOLDSTEIN; W E KNOX; E J BEHRMAN
Journal:  J Biol Chem       Date:  1962-09       Impact factor: 5.157

2.  A block in glycine cleavage reaction as a common mechanism in ketotic and nonketotic hyperglycinemia.

Authors:  K Tada
Journal:  Pediatr Res       Date:  1974-07       Impact factor: 3.756

3.  The oxidation of glycine and propionic acid in propionic acidemia with ketotic hyperglycinemia.

Authors:  T Ando; W L Nyhan; J D Connor; K Rasmussen; G Donnell; N Barnes; D Cottom; D Hull
Journal:  Pediatr Res       Date:  1972-06       Impact factor: 3.756

4.  Hyperglycinemia: a defect in glycine cleavage reaction.

Authors:  K Tada; K Narisawa; T Yoshida; T Konno; Y Yokoyama
Journal:  Tohoku J Exp Med       Date:  1969-07       Impact factor: 1.848

5.  Modified methods for the determination of carbamyl aspartate.

Authors:  L M Prescott; M E Jones
Journal:  Anal Biochem       Date:  1969-12       Impact factor: 3.365

6.  Serine transhydroxymethylase. Identification as the threonine and allothreonine aldolases.

Authors:  L Schirch; T Gross
Journal:  J Biol Chem       Date:  1968-11-10       Impact factor: 5.157

7.  Evidence of defective threonine metabolism in non-ketotic hyperglycinaemia.

Authors:  I Krieger; M Nigro
Journal:  J Inherit Metab Dis       Date:  1983       Impact factor: 4.982

8.  L-Threonine administration increases glycine concentrations in the rat central nervous system.

Authors:  T J Maher; R J Wurtman
Journal:  Life Sci       Date:  1980-04-21       Impact factor: 5.037

9.  Studies of the glycine metabolism in a patient with D-glyceric acidemia and hyperglycinemia.

Authors:  S Kølvraa; E Christensen; N J Brandt
Journal:  Pediatr Res       Date:  1980-09       Impact factor: 3.756

10.  Studies of the glycine cleavage enzyme system in brain from infants with glycine encephalopathy.

Authors:  T L Perry; N Urquhart; S Hansen
Journal:  Pediatr Res       Date:  1977-12       Impact factor: 3.756
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  3 in total

Review 1.  Non-ketotic hyperglycinaemia: clinical and biochemical aspects.

Authors:  K Tada; K Hayasaka
Journal:  Eur J Pediatr       Date:  1987-05       Impact factor: 3.183

2.  Crystal structure of the pyridoxal-5'-phosphate-dependent serine dehydratase from human liver.

Authors:  Lei Sun; Mark Bartlam; Yiwei Liu; Hai Pang; Zihe Rao
Journal:  Protein Sci       Date:  2005-02-02       Impact factor: 6.725

Review 3.  The biochemical basis of mitochondrial diseases.

Authors:  H R Scholte
Journal:  J Bioenerg Biomembr       Date:  1988-04       Impact factor: 2.945
  3 in total

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