Literature DB >> 6483508

The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease.

S E Snyderman, F Goldstein, C Sansaricq, P M Norton.   

Abstract

Plasma branched chain amino acid levels and their respective ketoacid analogues were determined in seven maple syrup urine disease patients ranging in age from 12 h to 12 years. One hundred one pairs were analyzed. There was a high degree of correlation between the amino acid and its ketoacid analogue at every amino acid level. The coefficient of correlation (0.84) was highest for leucine-alpha-ketoisocaproic acid. The ratio of ketoacid analogue to amino acid, (0.87), was also the greatest for leucine. The close correlation implies that adequate monitoring for therapy can be accomplished by the use of the technically simpler and more rapid determination of the plasma branched chain amino acids.

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Year:  1984        PMID： 6483508     DOI： 10.1203/00006450-198409000-00009

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


  10 in total

1.  Alpha-ketoisocaproic acid increases phosphorylation of intermediate filament proteins from rat cerebral cortex by mechanisms involving Ca2+ and cAMP.

Authors:  Cláudia Funchal; Ariane Zamoner; André Quincozes dos Santos; Samanta Oliveira Loureiro; Moacir Wajner; Regina Pessoa-Pureur
Journal:  Neurochem Res       Date:  2005-09       Impact factor: 3.996

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.  The diagnosis and management of MSUD in Saudi Arabia by using two different methods.

Authors:  S B Subramanyam; S M Qadri; M B Dhalla; P T Ozand
Journal:  Indian J Pediatr       Date:  1990 Sep-Oct       Impact factor: 1.967

4.  alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats.

Authors:  Raquel Bridi; César A Braun; Giovanni K Zorzi; Clóvis M D Wannmacher; Moacir Wajner; Eduardo G Lissi; Carlos Severo Dutra-Filho
Journal:  Metab Brain Dis       Date:  2005-06       Impact factor: 3.584

5.  Branched chain amino acids induce apoptosis in neural cells without mitochondrial membrane depolarization or cytochrome c release: implications for neurological impairment associated with maple syrup urine disease.

Authors:  P Jouvet; P Rustin; D L Taylor; J M Pocock; U Felderhoff-Mueser; N D Mazarakis; C Sarraf; U Joashi; M Kozma; K Greenwood; A D Edwards; H Mehmet
Journal:  Mol Biol Cell       Date:  2000-05       Impact factor: 4.138

6.  Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease.

Authors:  P Schadewaldt; H W Hammen; A C Ott; U Wendel
Journal:  J Inherit Metab Dis       Date:  1999-08       Impact factor: 4.982

7.  Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex.

Authors:  André Wajner; Cristiane Bürger; Carlos Severo Dutra-Filho; Moacir Wajner; Angela Terezinha de Souza Wyse; Clóvis Milton Duval Wannmacher
Journal:  Metab Brain Dis       Date:  2007-03       Impact factor: 3.584

8.  Stimulation of lipid peroxidation in vitro in rat brain by the metabolites accumulating in maple syrup urine disease.

Authors:  Fernanda U Fontella; Edson Gassen; Vânia Pulrolnik; Clóvis M D Wannmacher; Adriane B Klein; Moacir Wajner; Carlos S Dutra-Filho
Journal:  Metab Brain Dis       Date:  2002-03       Impact factor: 3.584

9.  ENU mutagenesis identifies mice with mitochondrial branched-chain aminotransferase deficiency resembling human maple syrup urine disease.

Authors:  Jer-Yuarn Wu; Hsiao-Jung Kao; Sing-Chung Li; Robert Stevens; Steven Hillman; David Millington; Yuan-Tsong Chen
Journal:  J Clin Invest       Date:  2004-02       Impact factor: 14.808

10.  Intellectual performance of children with maple syrup urine disease.

Authors:  C Hilliges; D Awiszus; U Wendel
Journal:  Eur J Pediatr       Date:  1993-02       Impact factor: 3.183
  10 in total

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