3-Ureidopropionate contributes to the neuropathology of 3-ureidopropionase deficiency and severe propionic aciduria: A Hypothesis

Journal of Neuroscience Research

Volumn 66, Issue 4, 2001, Pages 666-673

  Kölker, Stefan ^a   Okun, Jürgen G ^a   Hörster, Friederike ^a   Assmann, Birgit ^a   Ahlemeyer, Barbara ^b   Kohlmüller, Dirk ^a   Exner Camps, Sonja ^a   Mayatepek, Ertan ^a   Krieglstein, Josef ^b   Hoffmann, Georg F ^a  

^a UNIVERSITY HOSPITAL HEIDELBERG   (Germany)

^b PHILIPPS UNIVERSITY MARBURG   (Germany)

Author keywords

Beta oxidation; Excitotoxicity; Inborn errors of metabolism; Reactive oxygen species; Respiratory chain

Indexed keywords

3 UREIDOPROPIONASE; 3 UREIDOPROPIONATE; 6 CYANO 7 NITRO 2,3 QUINOXALINEDIONE; ALPHA TOCOPHEROL; DIZOCILPINE; GLUTAMATE RECEPTOR ANTAGONIST; N METHYL DEXTRO ASPARTIC ACID RECEPTOR BLOCKING AGENT; PROPIONIC ACID DERIVATIVE; SUCCINIC ACID; UNCLASSIFIED DRUG;

3 UREIDOPROPIONASE DEFICIENCY; ANIMAL CELL; ARTICLE; CALCIUM CELL LEVEL; CELL CULTURE; CHICKEN; CONTROLLED STUDY; DYSKINESIA; ELECTRON TRANSPORT; ENZYME DEFICIENCY; FATTY ACID OXIDATION; MITOCHONDRIAL ENERGY TRANSFER; NERVE CELL; NERVE CELL LESION; NEUROPATHOLOGY; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROPIONIC ACIDURIA; PYRIMIDINE METABOLISM; RESPIRATORY CHAIN;

ANIMALS; BETA-ALANINE; BRAIN DISEASES, METABOLIC; CALCIUM; CELL DEATH; CELLS, CULTURED; CENTRAL NERVOUS SYSTEM; CHICK EMBRYO; CITRULLINE; ELECTRON TRANSPORT; ENERGY METABOLISM; ENZYME INHIBITORS; EXCITATORY AMINO ACID ANTAGONISTS; FORMAMIDES; GLUTAMIC ACID; MITOCHONDRIA; NEURONS; NEUROTOXINS; OXIDATIVE STRESS; PROPIONIC ACIDS; REACTIVE OXYGEN SPECIES; RECEPTORS, N-METHYL-D-ASPARTATE; UREA;

EID: 0035889487     PISSN: 03604012     EISSN: None     Source Type: Journal    
DOI: 10.1002/jnr.10012     Document Type: Article

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