SIL1, the endoplasmic-reticulum-localized BiP co-chaperone, plays a crucial role in maintaining skeletal muscle proteostasis and physiology
Mutations in SIL1, a cofactor for the endoplasmic reticulum (ER)-localized Hsp70 chaperone, BiP, cause Marinesco-Sjögren syndrome (MSS), an autosomal recessive disorder. Using a mouse model, we characterized molecular aspects of the progressive myopathy associated with MSS. Proteomic profiling of q...
Guardado en:
Autores principales: | Viraj P. Ichhaporia (Autor), Jieun Kim (Autor), Kanisha Kavdia (Autor), Peter Vogel (Autor), Linda Horner (Autor), Sharon Frase (Autor), Linda M. Hendershot (Autor) |
---|---|
Formato: | Libro |
Publicado: |
The Company of Biologists,
2018-05-01T00:00:00Z.
|
Materias: | |
Acceso en línea: | Connect to this object online. |
Etiquetas: |
Agregar Etiqueta
Sin Etiquetas, Sea el primero en etiquetar este registro!
|
Ejemplares similares
-
Endoplasmic Reticulum Stress-Associated Chaperones, Bip/GRP78 and Calnexin are Overexpressed in Keratocystic Odontogenic Tumours
por: Maria Pavli, et al.
Publicado: (2014) -
The molecular chaperone GRP78/BiP in the development of chemoresistance: mechanism and possible treatment
por: Corinna eRoller, et al.
Publicado: (2013) -
Editorial: Restoring endoplasmic reticulum proteostasis to treat neurological disorders
por: Danilo B. Medinas, et al.
Publicado: (2023) -
Pharmacological chaperones restore proteostasis of epilepsy-associated GABAA receptor variants
por: Ya-Juan Wang, et al.
Publicado: (2024) -
Chemical Chaperones to Inhibit Endoplasmic Reticulum Stress: Implications in Diseases
por: Jeon JH, et al.
Publicado: (2022)