Literature DB >> 1770796

Alternative methionine degradation via the transamination pathway: an option for therapy for homocystinuria due to cystathionine synthase deficiency.

H J Blom1, G H Boers, A Tangerman, W A Gahl, J M Trijbels.   

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Year:  1991        PMID: 1770796     DOI: 10.1007/bf01811707

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


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  6 in total

1.  Transsulfuration in an adult with hepatic methionine adenosyltransferase deficiency.

Authors:  W A Gahl; I Bernardini; J D Finkelstein; A Tangerman; J J Martin; H J Blom; K D Mullen; S H Mudd
Journal:  J Clin Invest       Date:  1988-02       Impact factor: 14.808

2.  Oxidative decarboxylation of 4-methylthio-2-oxobutyrate by branched-chain 2-oxo acid dehydrogenase complex.

Authors:  S M Jones; S J Yeaman
Journal:  Biochem J       Date:  1986-07-15       Impact factor: 3.857

3.  Methanethiol and dimethylsulfide formation from 3-methylthiopropionate in human and rat hepatocytes.

Authors:  H J Blom; J P van den Elzen; S H Yap; A Tangerman
Journal:  Biochim Biophys Acta       Date:  1988-11-18

Review 4.  Deficiency of the alpha and beta subunits of pyruvate dehydrogenase in a patient with lactic acidosis and unexpected sudden death.

Authors:  W Sperl; W Ruitenbeek; C M Kerkhof; R C Sengers; J M Trijbels; J P Guggenbichler; A J Janssen; J A Bakkeren
Journal:  Eur J Pediatr       Date:  1990-04       Impact factor: 3.183

5.  Cystathionine-synthase-deficient patients do not use the transamination pathway of methionine to reduce hypermethioninemia and homocystinemia.

Authors:  H J Blom; G H Boers; J M Trijbels; J J van Roessel; A Tangerman
Journal:  Metabolism       Date:  1989-06       Impact factor: 8.694

6.  Transamination of methionine in humans.

Authors:  H J Blom; G H Boers; J P van den Elzen; W A Gahl; A Tangerman
Journal:  Clin Sci (Lond)       Date:  1989-01       Impact factor: 6.124

  6 in total
  1 in total

1.  Methionine transamination--metabolic function and subcellular compartmentation.

Authors:  P W Scislowski; K Pickard
Journal:  Mol Cell Biochem       Date:  1993-12-08       Impact factor: 3.396

  1 in total

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