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Literature DB >> 17574538

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

Abstract

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.

Entities: Chemical Disease Species

Mesh：

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Year: 2007 PMID： 17574538 DOI： 10.1007/s10545-007-0562-6

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

88 in total

Review 1. Rho proteins: linking signaling with membrane trafficking.

Authors: A J Ridley
Journal: Traffic Date: 2001-05 Impact factor: 6.215

Review 2. Intermediate filaments: molecular structure, assembly mechanism, and integration into functionally distinct intracellular Scaffolds.

Authors: Harald Herrmann; Ueli Aebi
Journal: Annu Rev Biochem Date: 2004 Impact factor: 23.643

Review 3. The co-workers of actin filaments: from cell structures to signals.

Authors: Céline Revenu; Rafika Athman; Sylvie Robine; Daniel Louvard
Journal: Nat Rev Mol Cell Biol Date: 2004-08 Impact factor: 94.444

4. Glutamate binding in the rat cerebral cortex during ontogeny.

Authors: C Sanderson; S Murphy
Journal: Brain Res Date: 1981-10 Impact factor: 3.252

5. Methylmalonic and propionic acids increase the in vitro incorporation of 32P into cytoskeletal proteins from cerebral cortex of young rats through NMDA glutamate receptors.

Authors: A de Mattos-Dutra; R Meirelles; B Bevilaqua da Rocha; T Kommers; S T Wofchuk; M Wajner; R Pessoa-Pureur
Journal: Brain Res Date: 2000-02-21 Impact factor: 3.252

6. Disruption of actin cytoskeleton in cultured rat astrocytes suppresses ATP- and bradykinin-induced [Ca(2+)](i) oscillations by reducing the coupling efficiency between Ca(2+) release, capacitative Ca(2+) entry, and store refilling.

Authors: M Sergeeva; J J Ubl; G Reiser
Journal: Neuroscience Date: 2000 Impact factor: 3.590

7. Maple syrup urine disease: findings on CT and MR scans of the brain in 10 infants.

Authors: J Brismar; A Aqeel; G Brismar; R Coates; G Gascon; P Ozand
Journal: AJNR Am J Neuroradiol Date: 1990 Nov-Dec Impact factor: 3.825

8. Effect of leucine administration on creatine kinase activity in rat brain.

Authors: Carmen Pilla; Rui Felipe de Oliveira Cardozo; Carlos Severo Dutra-Filho; Angela Terezinha Souza Wyse; Moacir Wajner; Clóvis Milton Duval Wannmacher
Journal: Metab Brain Dis Date: 2003-03 Impact factor: 3.584

9. Glutamate and gamma-aminobutyric acid neurotransmitter systems in the acute phase of maple syrup urine disease and citrullinemia encephalopathies in newborn calves.

Authors: P R Dodd; S H Williams; A L Gundlach; P A Harper; P J Healy; J A Dennis; G A Johnston
Journal: J Neurochem Date: 1992-08 Impact factor: 5.372

10. Effect of propionic and methylmalonic acids on the in vitro phosphorylation of intermediate filaments from cerebral cortex of rats during development.

Authors: Lúcia Maria Vieira de Almeida; Cláudia Funchal; Priscila de Lima Pelaez; Franciele Dall Bello Pessutto; Samanta Oliveira Loureiro; Lilian Vivian; Moacir Wajner; Regina Pessoa-Pureur
Journal: Metab Brain Dis Date: 2003-09 Impact factor: 3.584

6 in total

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

Review 1. Rho proteins: linking signaling with membrane trafficking.

Review 2. Intermediate filaments: molecular structure, assembly mechanism, and integration into functionally distinct intracellular Scaffolds.

Review 3. The co-workers of actin filaments: from cell structures to signals.

4. Glutamate binding in the rat cerebral cortex during ontogeny.

5. Methylmalonic and propionic acids increase the in vitro incorporation of 32P into cytoskeletal proteins from cerebral cortex of young rats through NMDA glutamate receptors.

6. Disruption of actin cytoskeleton in cultured rat astrocytes suppresses ATP- and bradykinin-induced [Ca(2+)](i) oscillations by reducing the coupling efficiency between Ca(2+) release, capacitative Ca(2+) entry, and store refilling.

7. Maple syrup urine disease: findings on CT and MR scans of the brain in 10 infants.

8. Effect of leucine administration on creatine kinase activity in rat brain.

9. Glutamate and gamma-aminobutyric acid neurotransmitter systems in the acute phase of maple syrup urine disease and citrullinemia encephalopathies in newborn calves.

10. Effect of propionic and methylmalonic acids on the in vitro phosphorylation of intermediate filaments from cerebral cortex of rats during development.

Review 1. Signal transduction in inherited metabolic disorders: a model for a possible pathogenetic mechanism.

Review 2. Cytoskeleton as a Target of Quinolinic Acid Neurotoxicity: Insight from Animal Models.

Review 3. In Vivo NMR Studies of the Brain with Hereditary or Acquired Metabolic Disorders.

Review 4. "Classical organic acidurias": diagnosis and pathogenesis.

5. The first use of N-carbamylglutamate in a patient with decompensated maple syrup urine disease.

Review 6. Signaling mechanisms and disrupted cytoskeleton in the diphenyl ditelluride neurotoxicity.