Peripheral nervous system manifestations in a Sandhoff disease mouse model: nerve conduction, myelin structure, lipid analysis

BACKGROUND: Sandhoff disease is an inherited lysosomal storage disease caused by a mutation in the gene for the β-subunit (Hexb gene) of β-hexosaminidase A (αβ) and B (ββ). The β-subunit together with the GM2 activator protein catabolize ganglioside GM2. This enzyme deficiency results in GM2 accumul...

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Detalles Bibliográficos
Autores principales: McNally, Melanie A, Baek, Rena C, Avila, Robin L, Seyfried, Thomas N, Strichartz, Gary R, Kirschner, Daniel A
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2007
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1976615/
https://www.ncbi.nlm.nih.gov/pubmed/17623103
http://dx.doi.org/10.1186/1477-5751-6-8

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