Literature DB >> 5014609

Cystathionine synthase activity in human lymphocytes: induction by phytohemagglutinin.

J L Goldstein, B K Campbell, S M Gartler.   

Abstract

Uncultured human leukocytes contain no detectable cystathionine synthase activity. A method is described in which the addition of phytohemagglutinin (PHA) to short-term lymphocyte cultures results in a significant induction of enzymatic activity. This PHA-stimulated activity has characteristics that resemble those previously described for cystathionine synthase of normal liver and cultured fibroblasts. Lymphocyte cystathionine synthase activity is completely dependent on the presence of homocysteine and is absent or severely deficient in extracts from individuals with the syndrome of homocystinuria. This system for induction of cystathionine synthase in lymphocytes thus provides a simple in vitro technique for (a) diagnosing homocystinuria, (b) studying the mechanism of enzyme regulation and differentiation, and (c) examining the nutritional and hormonal control of cystathionine synthase activity both in normal subjects and homocystinuric patients.

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Year:  1972        PMID: 5014609      PMCID: PMC302215          DOI: 10.1172/JCI106863

Source DB:  PubMed          Journal:  J Clin Invest        ISSN: 0021-9738            Impact factor:   14.808


  7 in total

1.  HOMOCYSTINURIA: AN ENZYMATIC DEFECT.

Authors:  S H MUDD; J D FINKELSTEIN; F IRREVERRE; L LASTER
Journal:  Science       Date:  1964-03-27       Impact factor: 47.728

2.  Protein measurement with the Folin phenol reagent.

Authors:  O H LOWRY; N J ROSEBROUGH; A L FARR; R J RANDALL
Journal:  J Biol Chem       Date:  1951-11       Impact factor: 5.157

3.  Transsulfuration in mammals. Microassays and tissue distributions of three enzymes of the pathway.

Authors:  S H Mudd; J D Finkelstein; F Irreverre; L Laster
Journal:  J Biol Chem       Date:  1965-11       Impact factor: 5.157

4.  A modified assay for cystathionine synthase.

Authors:  P Manowitz; J Racevskis; D G Gilmour
Journal:  Clin Chim Acta       Date:  1970-05       Impact factor: 3.786

5.  Homocystinuria due to cystathionine synthase deficiency: the effect of pyridoxine.

Authors:  S H Mudd; W A Edwards; P M Loeb; M S Brown; L Laster
Journal:  J Clin Invest       Date:  1970-09       Impact factor: 14.808

6.  Cystathionine synthase in tissue culture derived from human skin: enzyme defect in homocystinuria.

Authors:  B W Uhlendorf; S H Mudd
Journal:  Science       Date:  1968-05-31       Impact factor: 47.728

7.  Homocystinuria due to cystathionine synthase deficiency.

Authors: 
Journal:  Ann Intern Med       Date:  1965-12       Impact factor: 25.391

  7 in total
  9 in total

1.  Enzyme studies in cystathione-beta-synthase deficiency: a possible effect of elevated intracellular levels of homocystine in kinetic studies.

Authors:  F Hemraj; R Griffiths
Journal:  J Inherit Metab Dis       Date:  1978       Impact factor: 4.982

2.  Reversal of UDP-galactose 4-epimerase deficiency of human leukocytes in culture.

Authors:  B Mitchell; E Haigis; B Steinmann; R Gitzelmann
Journal:  Proc Natl Acad Sci U S A       Date:  1975-12       Impact factor: 11.205

3.  The molecular basis of cystathionine beta-synthase deficiency in Dutch patients with homocystinuria: effect of CBS genotype on biochemical and clinical phenotype and on response to treatment.

Authors:  L A Kluijtmans; G H Boers; J P Kraus; L P van den Heuvel; J R Cruysberg; F J Trijbels; H J Blom
Journal:  Am J Hum Genet       Date:  1999-07       Impact factor: 11.025

4.  Dynamic change of hydrogen sulfide after traumatic brain injury and its effect in mice.

Authors:  Mingyang Zhang; Haiyan Shan; Tao Wang; Weili Liu; Yaoqi Wang; Long Wang; Lu Zhang; Pan Chang; Wenwen Dong; Xiping Chen; Luyang Tao
Journal:  Neurochem Res       Date:  2013-01-17       Impact factor: 3.996

5.  Homocystinuria: heterozygote detection using phytohemagglutinin-stimulated lymphocytes.

Authors:  J L Goldstein; B K Campbell; S M Gartler
Journal:  J Clin Invest       Date:  1973-01       Impact factor: 14.808

6.  Homocystinuria. Evidence for three distinct classes of cystathionine beta-synthase mutants in cultured fibroblasts.

Authors:  B Fowler; J Kraus; S Packman; L E Rosenberg
Journal:  J Clin Invest       Date:  1978-03       Impact factor: 14.808

7.  Homocystinuria: studies on cystathionine beta-synthase, S-adenosylmethionine synthetase and cystathionase activities in skin fibroblasts.

Authors:  A H Bittles; N A Carson
Journal:  J Inherit Metab Dis       Date:  1981       Impact factor: 4.982

8.  Effect of chronologic age on induction of cystathionine synthase, uroporphyrinogen I synthase, and glucose-6-phosphate dehydrogenase activities in lymphocytes.

Authors:  S M Gartler; S K Hornung; A G Motulsky
Journal:  Proc Natl Acad Sci U S A       Date:  1981-03       Impact factor: 11.205

9.  Methionine-deficient diet induces post-transcriptional downregulation of cystathionine β-synthase.

Authors:  Baiqing Tang; Aladdin Mustafa; Sapna Gupta; Stepan Melnyk; S Jill James; Warren D Kruger
Journal:  Nutrition       Date:  2009-12-29       Impact factor: 4.008

  9 in total

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