Cargando…
Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease
To study the neuronal deficits in neuronopathic Gaucher Disease (nGD), the chronological behavioral profiles and the age of onset of brain abnormalities were characterized in a chronic nGD mouse model (9V/null). Progressive accumulation of glucosylceramide (GC) and glucosylsphingosine (GS) in the br...
Autores principales: | Dai, Mei, Liou, Benjamin, Swope, Brittany, Wang, Xiaohong, Zhang, Wujuan, Inskeep, Venette, Grabowski, Gregory A., Sun, Ying, Pan, Dao |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2016
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5012639/ https://www.ncbi.nlm.nih.gov/pubmed/27598339 http://dx.doi.org/10.1371/journal.pone.0162367 |
Ejemplares similares
-
Isofagomine In Vivo Effects in a Neuronopathic Gaucher Disease Mouse
por: Sun, Ying, et al.
Publicado: (2011) -
Systemic enzyme delivery by blood-brain barrier-penetrating SapC-DOPS nanovesicles for treatment of neuronopathic Gaucher disease
por: Sun, Ying, et al.
Publicado: (2020) -
CNS-accessible Inhibitor of Glucosylceramide Synthase for Substrate Reduction Therapy of Neuronopathic Gaucher Disease
por: Marshall, John, et al.
Publicado: (2016) -
Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits
por: Sun, Ying, et al.
Publicado: (2010) -
The definition of neuronopathic Gaucher disease
por: Schiffmann, Raphael, et al.
Publicado: (2020)