Behavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV

Mucolipidosis IV (MLIV) is caused by mutations in the gene MCOLN1. Patients with MLIV have severe neurologic deficits and very little is known about the brain pathology in this lysosomal disease. Using an accurate mouse model of mucolipidosis IV, we observed early behavioral deficits which were acco...

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Detalles Bibliográficos
Autores principales: Grishchuk, Yulia, Sri, Sarmi, Rudinskiy, Nikita, Ma, Weiyuan, Stember, Katherine G., Cottle, Matthew W., Sapp, Ellen, Difiglia, Marian, Muzikansky, Alona, Betensky, Rebecca A., Wong, Andrew M. S., Bacskai, Brian J., Hyman, Bradley T., Kelleher, Raymond J., Cooper, Jonathan D., Slaugenhaupt, Susan A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4173007/
https://www.ncbi.nlm.nih.gov/pubmed/25200117
http://dx.doi.org/10.1186/s40478-014-0133-7

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4173007/
https://www.ncbi.nlm.nih.gov/pubmed/25200117
http://dx.doi.org/10.1186/s40478-014-0133-7

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