Literature DB >> 2919111

Interrelation between the metabolism of L-isoleucine and L-allo-isoleucine in patients with maple syrup urine disease.

U Wendel1, U Langenbeck, J W Seakins.   

Abstract

The nonprotein amino acid L-allo-isoleucine is formed endogenously in maple syrup urine disease patients from (R)-3-methyl-2-oxo-pentanoic acid. During strict metabolic balance, the plasma L-allo-isoleucine/L-isoleucine ratio correlates inversely with the residual activity of the branched-chain 2-oxoacid dehydrogenase in fibroblasts and thus constitutes a relevant in vivo parameter of the severity of the metabolic defect in MSUD patients.

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Year:  1989        PMID： 2919111     DOI： 10.1203/00006450-198901000-00003

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


  6 in total

1.  The altered human serum metabolome induced by a marathon.

Authors:  Zinandré Stander; Laneke Luies; Lodewyk J Mienie; Karen M Keane; Glyn Howatson; Tom Clifford; Emma J Stevenson; Du Toit Loots
Journal:  Metabolomics       Date:  2018-11-03       Impact factor: 4.290

2.  Maple syrup urine disease: interrelations between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination.

Authors:  E Treacy; C L Clow; T R Reade; D Chitayat; O A Mamer; C R Scriver
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

3.  Variant maple syrup urine disease (MSUD)--the entire spectrum.

Authors:  E Simon; N Flaschker; P Schadewaldt; U Langenbeck; U Wendel
Journal:  J Inherit Metab Dis       Date:  2006-10-25       Impact factor: 4.982

4.  On the mechanism of L-alloisoleucine formation: studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease.

Authors:  P Schadewaldt; H W Hammen; C Dalle-Feste; U Wendel
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

5.  d-Amino Acid Levels in Perfused Mouse Brain Tissue and Blood: A Comparative Study.

Authors:  Choyce A Weatherly; Siqi Du; Curran Parpia; Polan T Santos; Adam L Hartman; Daniel W Armstrong
Journal:  ACS Chem Neurosci       Date:  2017-02-16       Impact factor: 4.418

6.  Sensory-motor polyneuropathy occurring in variant maple syrup urine disease.

Authors:  S Harty; M D King; B McCoy; D Costigan; E P Treacy
Journal:  J Inherit Metab Dis       Date:  2008-10-16       Impact factor: 4.982
  6 in total

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