Cargando…
Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice
Saposin B derives from the multi-functional precursor, prosaposin, and functions as an activity enhancer for several glycosphingolipid (GSL) hydrolases. Mutations in saposin B present in humans with phenotypes resembling metachromatic leukodystrophy. To gain insight into saposin B's physiologic...
Autores principales: | Sun, Ying, Witte, David P., Ran, Huimin, Zamzow, Matt, Barnes, Sonya, Cheng, Hua, Han, Xianlin, Williams, Michael T., Skelton, Matthew R., Vorhees, Charles V., Grabowski, Gregory A. |
---|---|
Formato: | Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2008
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2465797/ https://www.ncbi.nlm.nih.gov/pubmed/18480170 http://dx.doi.org/10.1093/hmg/ddn135 |
Ejemplares similares
-
Specific saposin C deficiency: CNS impairment and acid β-glucosidase effects in the mouse
por: Sun, Ying, et al.
Publicado: (2010) -
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) -
Temporal gene expression profiling reveals CEBPD as a candidate regulator of brain disease in prosaposin deficient mice
por: Sun, Ying, et al.
Publicado: (2008) -
Combination of acid β-glucosidase mutation and Saposin C deficiency in mice reveals Gba1 mutation dependent and tissue-specific disease phenotype
por: Liou, Benjamin, et al.
Publicado: (2019) -
Structural features of many circular and leaderless bacteriocins are similar to those in saposins and saposin-like peptides
por: Towle, K. M., et al.
Publicado: (2017)