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Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: Insight from animal studies
Author(s) -
PessoaPureur R.,
Wajner M.
Publication year - 2007
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-007-0562-6
Subject(s) - maple syrup urine disease , neuropathology , urine , maple , disease , medicine , biology , biochemistry , amino acid , botany , leucine
Summary In this short review we provide evidence that the branched‐chain keto acids accumulating in the neurometabolic disorder maple syrup urine disease disturb rat cerebral cytoskeleton in a developmentally regulated manner. Alterations of protein phosphorylation leading to brain cytoskeletal misregulation and neural cell death caused by these metabolites are associated with energy deprivation, oxidative stress and excitotoxicity that may ultimately disrupt normal cell function and viability.