- Tytuł:
-
Regulation of ClC-2
Chloride Channel Proteostasis by Molecular Chaperones: Correction of Leukodystrophy-Associated Defect. - Autorzy:
- Źródło:
- International journal of molecular sciences [Int J Mol Sci] 2021 May 30; Vol. 22 (11). Date of Electronic Publication: 2021 May 30.
- Typ publikacji:
- Journal Article
- MeSH Terms:
-
Brain/*metabolism
Chloride Channels /*genetics
Leydig Cells/*metabolismNeurons /*metabolism
Pelizaeus-Merzbacher Disease/*genetics
Proteostasis/*genetics
Animals ; Benzoquinones/pharmacology ; Brain/drug effects ; Brain/pathology ; CHO Cells ; CLC-2Chloride Channels ;Chloride Channels /deficiency ; Cricetulus ; Disease Models, Animal ; Endoplasmic Reticulum-Associated Degradation/drug effects ; Gene Expression Regulation ; HEK293 Cells ; HSP70 Heat-Shock Proteins/genetics ; HSP70 Heat-Shock Proteins/metabolism ; HSP90 Heat-Shock Proteins/genetics ; HSP90 Heat-Shock Proteins/metabolism ; Humans ; Lactams, Macrocyclic/pharmacology ; Leydig Cells/drug effects ; Leydig Cells/pathology ; Male ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Molecular Chaperones/genetics ; Molecular Chaperones/metabolism ;Neurons /drug effects ;Neurons /pathology ; Pelizaeus-Merzbacher Disease/drug therapy ; Pelizaeus-Merzbacher Disease/metabolism ; Pelizaeus-Merzbacher Disease/pathology ; Protein Isoforms/deficiency ; Protein Isoforms/genetics ; Signal Transduction ; Tacrolimus Binding Proteins/genetics ; Tacrolimus Binding Proteins/metabolism
Czasopismo naukowe