Literature DB >> 13775398

Inhibition of brain glutamic acid decarboxylase by phenylalanine, valine, and leucine derivatives: a suggestion concerning the etiology of the neurological defect in phenylketonuria and branched-chain ketonuria.

R E TASHIAN.   

Abstract

Entities:  

Keywords:  AMINO ACIDS/pharmacology; BRAIN/metabolism; DESMOLASES/antagonists; MENTAL DEFICIENCY/etiology; METABOLIC DISEASES/etiology; PHENYLPYRUVIC OLIGOPHRENIA/etiology

Mesh：

Substances：

Year:  1961        PMID： 13775398

Source DB:  PubMed          Journal:  Metabolism        ISSN： 0026-0495            Impact factor:   8.694


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  18 in total

1.  Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.

Authors:  Cláudia Funchal; Patrícia Fernanda Schuck; André Quincozes Dos Santos; Maria Caroline Jacques-Silva; Carmem Gottfried; Regina Pessoa-Pureur; Moacir Wajner
Journal:  Cell Mol Neurobiol       Date:  2006-02       Impact factor: 5.046

2.  Importance of choline during growth, with particular reference to synthetic diets in phenylketonuria.

Authors:  K M WILSON; B E CLAYTON
Journal:  Arch Dis Child       Date:  1962-12       Impact factor: 3.791

3.  A chemical investigation of the defects of myelination in phenylketonuria.

Authors:  L CROME; V TYMMS; L I WOOLF
Journal:  J Neurol Neurosurg Psychiatry       Date:  1962-05       Impact factor: 10.154

4.  Effect of alpha-ketoisocaproate and leucine on the in vivo oxidation of glutamate and glutamine in the rat brain.

Authors:  H R Zielke; Y Huang; P J Baab; R M Collins; C L Zielke; J T Tildon
Journal:  Neurochem Res       Date:  1997-09       Impact factor: 3.996

5.  Abnormal amino acid metabolism and brain protein synthesis during neural development.

Authors:  J V Hughes; T C Johnson
Journal:  Neurochem Res       Date:  1978-08       Impact factor: 3.996

6.  Maple syrup urine disease: An uncommon cause for neonatal metabolic distress.

Authors:  R Christopher; S V Babu; L Nirmala; G R Rangaswamy; C P Narayan; K T Shetty
Journal:  Indian J Clin Biochem       Date:  1999-07

7.  Phenylketonuria.

Authors:  B E Clayton
Journal:  J Med Genet       Date:  1971-03       Impact factor: 6.318

8.  Morphological alterations and cell death provoked by the branched-chain alpha-amino acids accumulating in maple syrup urine disease in astrocytes from rat cerebral cortex.

Authors:  Cláudia Funchal; Carmem Gottfried; Lúcia Maria Vieira de Almeida; André Quincozes dos Santos; Moacir Wajner; Regina Pessoa-Pureur
Journal:  Cell Mol Neurobiol       Date:  2005-08       Impact factor: 5.046

Review 9.  Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: insight from animal studies.

Authors:  R Pessoa-Pureur; M Wajner
Journal:  J Inherit Metab Dis       Date:  2007-06-14       Impact factor: 4.982

10.  Maple syrup urine disease: branched-chain amino acid concentrations and metabolism in cultured human lymphoblasts.

Authors:  S D Skaper; D P Molden; J E Seegmiller
Journal:  Biochem Genet       Date:  1976-08       Impact factor: 1.890
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