The unsolved puzzle of neuropathogenesis in glutaric aciduria type I

Molecular Genetics and Metabolism

Volumn 104, Issue 4, 2011, Pages 425-437

  Jafari, Paris ^a   Braissant, Olivier ^a   Bonafé, Luisa ^a   Ballhausen, Diana ^a  

^a LAUSANNE UNIVERSITY HOSPITAL   (Switzerland)

Author keywords

Energy failure; Excitotoxicity; Glutaric aciduria type I; Glutaryl CoA dehydrogenase; Neuropathogenesis; Oxidative stress

Indexed keywords

6 CYANO 7 NITRO 2,3 QUINOXALINEDIONE; AMINOGUANIDINE; CREATININE; DIZOCILPINE; IFENPRODIL; INTERLEUKIN 1BETA ANTIBODY; MUSCIMOL; N(G) NITROARGININE METHYL ESTER; PROBENECID; TAUROCHOLIC ACID;

BLOOD BRAIN BARRIER; BODY FLUID; BRAIN DISEASE; BRAIN NECROSIS; CATABOLISM; CLINICAL FEATURE; CONVULSION; DYSKINESIA; DYSTONIA; ENERGY METABOLISM; EXCITOTOXICITY; HUMAN; IN VITRO STUDY; IN VIVO STUDY; MULTIPLE ACYL COA DEHYDROGENASE DEFICIENCY; NERVE CELL LESION; NERVE DEGENERATION; NEUROPATHOLOGY; NONHUMAN; OXIDATIVE STRESS; PRIORITY JOURNAL; REVIEW; SEIZURE;

AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; BRAIN DISEASES, METABOLIC; DISEASE MODELS, ANIMAL; ENERGY METABOLISM; EXCITATORY AMINO ACID AGENTS; GLUTARYL-COA DEHYDROGENASE; HUMANS; ORGAN SPECIFICITY; OXIDATIVE STRESS; SYNAPTIC TRANSMISSION;

EID: 82255191631     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.08.027     Document Type: Review

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