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Gene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory response

BACKGROUND: The infantile form of neuronal ceroid lipofuscinosis (also known as infantile Batten disease) is caused by hereditary deficiency of a lysosomal enzyme, palmitoyl-protein thioesterase-1 (PPT1), and is characterized by severe cortical degeneration with blindness and cognitive and motor dys...

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Detalles Bibliográficos
Autores principales: Qiao, Xingwen, Lu, Jui-Yun, Hofmann, Sandra L
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2007
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2204004/
https://www.ncbi.nlm.nih.gov/pubmed/18021406
http://dx.doi.org/10.1186/1471-2202-8-95