Literature DB >> 198730

The activity of galactose-1-phosphate uridyltransferase and galactokinase in human fetal organs.

Y S Shin-Buehring, T Beier, A Tan, M Osang, J Schaub.   

Abstract

The activity of galactose-1-phosphate uridyltransferase (transferase) and galactokinase in several organs from human fetuses 7-28 weeks old was measured by using radioactive substrates and column chromatography for product identification. The specific activity of transferase and galactokinase increased with gestational age and reached, at the 28th week, a maximal level of 30.0 and 7.9 nmol/min/mg protein (units) in liver, 4.7 and 2.5 units in kidney, 6.0 and 4.0 units in lung, 6.7 and 2.9 units in spleen, 5.2 and 2.6 units in cardiac muscle, and 4.0 and 1.4 units in skeletal muscle, respectively. The activity in brain, on the other hand, remained quite constant with 1.2 units in the case of transferase and 0.5 units in the case of galactokinase during this period. The activities of both enzymes in the liver of children were slightly lower than the highest fetal level during the period of pregnancy studied. Galactokinase activity in fetal erythrocytes was approximately 4 times higher and the transferase activity approximately 30% higher than in adults. The Km value of fetal liver transferase for galactose-1-phosphate was found to be 0.330-0.357 mM and that of galactokinase for galactose, 0.265-0.277 mM.

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Year:  1977        PMID: 198730     DOI: 10.1203/00006450-197710000-00004

Source DB:  PubMed          Journal:  Pediatr Res        ISSN: 0031-3998            Impact factor:   3.756


  13 in total

1.  Age-dependency of galactose elimination capacity in healthy children and children with chronic liver disease.

Authors:  Aksel Lange; Henning Grønbæk; Hendrik Vilstrup; Susanne Keiding
Journal:  Scand J Gastroenterol       Date:  2010-11-01       Impact factor: 2.423

2.  Leukocytes as a diagnostic tool for classical galactosaemia.

Authors:  J M van der Klei-van Moorsel; M Brockstedt; J F Samsom; C Jakobs; R J Wanders
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982

3.  Metabolism of Mannose in Cultured Primary Rat Neurons.

Authors:  Wiebke Rastedt; Eva-Maria Blumrich; Ralf Dringen
Journal:  Neurochem Res       Date:  2017-03-27       Impact factor: 3.996

4.  Characteristics of galactokinase and galactose-1-phosphate uridyltransferase in cultivated fibroblasts and amniotic fluid cells.

Authors:  Y Shin-Buehring; H Leitner; H Henseleit; A Wirtz; B Haas; J Schaub
Journal:  Hum Genet       Date:  1979-04-17       Impact factor: 4.132

5.  Impaired hexose uptake by diploid skin fibroblasts from galactosaemic patients. Connection with cell growth and amino acid metabolism, and possible bearing on late-onset clinical symptoms.

Authors:  C Wolfrom; N Raynaud; N Kadhom; J Poggi; T Soni; M Gautier
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

6.  A founder mutation in the GK1 gene is responsible for galactokinase deficiency in Roma (Gypsies).

Authors:  L Kalaydjieva; A Perez-Lezaun; D Angelicheva; S Onengut; D Dye; N U Bosshard; A Jordanova; A Savov; P Yanakiev; I Kremensky; B Radeva; J Hallmayer; A Markov; V Nedkova; I Tournev; L Aneva; R Gitzelmann
Journal:  Am J Hum Genet       Date:  1999-11       Impact factor: 11.025

7.  The molecular basis of transferase galactosaemia in South African negroids.

Authors:  N Manga; T Jenkins; H Jackson; D A Whittaker; A B Lane
Journal:  J Inherit Metab Dis       Date:  1999-02       Impact factor: 4.982

8.  Clinical and biochemical evidence of skeletal muscle involvement in galactose-1-phosphate uridyl transferase deficiency.

Authors:  N Bresolin; G P Comi; F Fortunato; G Meola; A Gallanti; A Tajana; M Velicogna; E F Gonano; P Ninfali; S Pifferi
Journal:  J Neurol       Date:  1993-05       Impact factor: 4.849

9.  Mevalonate kinase assay using DEAE-cellulose column chromatography for first-trimester prenatal diagnosis and complementation analysis in mevalonic aciduria.

Authors:  G F Hoffmann; S U Brendel; S R Scharfschwerdt; Y S Shin; I M Speidel; K M Gibson
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

10.  Long-term outcome in 134 patients with galactosaemia.

Authors:  S Schweitzer; Y Shin; C Jakobs; J Brodehl
Journal:  Eur J Pediatr       Date:  1993-01       Impact factor: 3.183

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