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Literature DB >> 1580959

Abnormal dendritic development in maple syrup urine disease.

A Kamei¹, S Takashima, F Chan, L E Becker.

Abstract

The neuropathology of a 6-year-old boy with maple syrup urine disease revealed spongy appearance of the white matter and marked edema of the brainstem. Golgi studies demonstrated aberrant orientation of neurons together with abnormalities of dendrites and dendritic spines. Similar changes were observed in a patient with dihydropteridine reductase deficiency. Disorders of amino acid metabolism may be associated with alterations in the terminal stages of neuronal migration and maturation.

Entities: Disease Species

Mesh：

Year: 1992 PMID： 1580959 DOI： 10.1016/0887-8994(92)90038-z

Source DB: PubMed Journal: Pediatr Neurol ISSN： 0887-8994 Impact factor: 3.372

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Cited

14 in total

1. Liver transplantation for classical maple syrup urine disease: long-term follow-up in 37 patients and comparative United Network for Organ Sharing experience.

Authors: George V Mazariegos; D Holmes Morton; Rakesh Sindhi; Kyle Soltys; Navdeep Nayyar; Geoffrey Bond; Diana Shellmer; Benjamin Shneider; Jerry Vockley; Kevin A Strauss
Journal: J Pediatr Date: 2011-08-11 Impact factor: 4.406

2. Coadministration of branched-chain amino acids and lipopolysaccharide causes matrix metalloproteinase activation and blood-brain barrier breakdown.

Authors: Giselli Scaini; Meline O S Morais; Leticia S Galant; Francieli Vuolo; Dhébora M Dall'Igna; Matheus A B Pasquali; Vitor M Ramos; Daniel P Gelain; Jose Claudio F Moreira; Patrícia F Schuck; Gustavo C Ferreira; Francisco G Soriano; Felipe Dal-Pizzol; Emilio L Streck
Journal: Mol Neurobiol Date: 2014-01-05 Impact factor: 5.590

3. Serum Markers of Neurodegeneration in Maple Syrup Urine Disease.

Authors: Giselli Scaini; Tássia Tonon; Carolina F Moura de Souza; Patricia F Schuk; Gustavo C Ferreira; Joao Seda Neto; Tatiana Amorin; Ida Vanessa D Schwartz; Emilio L Streck
Journal: Mol Neurobiol Date: 2016-09-22 Impact factor: 5.590

Review 4. 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

Abnormal dendritic development in maple syrup urine disease.

1. Liver transplantation for classical maple syrup urine disease: long-term follow-up in 37 patients and comparative United Network for Organ Sharing experience.

2. Coadministration of branched-chain amino acids and lipopolysaccharide causes matrix metalloproteinase activation and blood-brain barrier breakdown.

3. Serum Markers of Neurodegeneration in Maple Syrup Urine Disease.

Review 4. Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: insight from animal studies.

5. Biochemical correlates of neuropsychiatric illness in maple syrup urine disease.

Review 6. The neuropsychiatry of inborn errors of metabolism.

7. Structural white matter changes in adolescents and young adults with maple syrup urine disease.

Review 8. Animal models of maple syrup urine disease.

9. Classical maple syrup urine disease and brain development: principles of management and formula design.

10. Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease.