Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease.
The neuropathic glycosphingolipidoses are a subgroup of lysosomal storage disorders for which there are no effective therapies. A potential approach is substrate reduction therapy using inhibitors of glucosylceramide synthase (GCS) to decrease the synthesis of glucosylceramide and related glycosphin...
Guardado en:
Autores principales: | Karen M Ashe, Dinesh Bangari, Lingyun Li, Mario A Cabrera-Salazar, Scott D Bercury, Jennifer B Nietupski, Christopher G F Cooper, Johannes M F G Aerts, Edward R Lee, Diane P Copeland, Seng H Cheng, Ronald K Scheule, John Marshall |
---|---|
Formato: | article |
Lenguaje: | EN |
Publicado: |
Public Library of Science (PLoS)
2011
|
Materias: | |
Acceso en línea: | https://doaj.org/article/cd3e8f6aa28047d9b5d2eaa2ee1c6e5d |
Etiquetas: |
Agregar Etiqueta
Sin Etiquetas, Sea el primero en etiquetar este registro!
|
Ejemplares similares
-
Correction of liver steatosis by a hydrophobic iminosugar modulating glycosphingolipids metabolism.
por: Elisa Lombardo, et al.
Publicado: (2012) -
Incorporation of Fluorescence Ceramide-Based HPLC Assay for Rapidly and Efficiently Assessing Glucosylceramide Synthase In Vivo
por: Sachin K. Khiste, et al.
Publicado: (2017) -
Neuronal expression of glucosylceramide synthase in central nervous system regulates body weight and energy homeostasis.
por: Viola Nordström, et al.
Publicado: (2013) -
Liver sphingomyelin synthase 1 deficiency causes steatosis, steatohepatitis, fibrosis, and tumorigenesis: An effect of glucosylceramide accumulation
por: Zhiqiang Li, et al.
Publicado: (2021) -
Iminosugar 1-Deoxynojirimycin (DNJ) sebagai Antiviral Infeksi Virus Dengue
por: Muhammad Luthfi Adnan
Publicado: (2020)