Calpain inhibitor and ibudilast rescue β cell functions in a cellular model of Wolfram syndrome.
Animals
Antineoplastic Agents
/ pharmacology
Calcium
/ metabolism
Calmodulin-Binding Proteins
/ genetics
Cell Survival
/ drug effects
Cysteine Proteinase Inhibitors
/ pharmacology
Disease Models, Animal
Endoplasmic Reticulum
/ metabolism
Gene Expression Regulation
Gene Knockout Techniques
Glycoproteins
Homeostasis
Hyperglycemia
Insulin Secretion
Insulin-Secreting Cells
/ drug effects
Membrane Proteins
/ genetics
Pyridines
/ pharmacology
Receptor, Insulin
Transcriptome
Wolfram Syndrome
/ drug therapy
Akt
calcium signaling
cell viability
diabetes
ibudilast
Journal
Proceedings of the National Academy of Sciences of the United States of America
ISSN: 1091-6490
Titre abrégé: Proc Natl Acad Sci U S A
Pays: United States
ID NLM: 7505876
Informations de publication
Date de publication:
21 07 2020
21 07 2020
Historique:
pubmed:
8
7
2020
medline:
15
9
2020
entrez:
8
7
2020
Statut:
ppublish
Résumé
Wolfram syndrome is a rare multisystem disease characterized by childhood-onset diabetes mellitus and progressive neurodegeneration. Most cases are attributed to pathogenic variants in a single gene, Wolfram syndrome 1 (
Identifiants
pubmed: 32632005
pii: 2007136117
doi: 10.1073/pnas.2007136117
pmc: PMC7382278
doi:
Substances chimiques
Antineoplastic Agents
0
Calmodulin-Binding Proteins
0
Cysteine Proteinase Inhibitors
0
Glycoproteins
0
Membrane Proteins
0
Pyridines
0
WFS1 protein, rat
0
calpain inhibitors
0
Receptor, Insulin
EC 2.7.10.1
ibudilast
M0TTH61XC5
Calcium
SY7Q814VUP
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
17389-17398Subventions
Organisme : NCATS NIH HHS
ID : UH3 TR002065
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR001863
Pays : United States
Organisme : NIDDK NIH HHS
ID : F30 DK111070
Pays : United States
Organisme : NIDDK NIH HHS
ID : P01 DK057751
Pays : United States
Organisme : NIDDK NIH HHS
ID : P60 DK020579
Pays : United States
Organisme : NIDDK NIH HHS
ID : P30 DK020579
Pays : United States
Organisme : NIDDK NIH HHS
ID : R01 DK112921
Pays : United States
Organisme : NCATS NIH HHS
ID : UH2 TR002065
Pays : United States
Déclaration de conflit d'intérêts
Competing interest statement: B.E.E. is a cofounder of Osmol Therapeutics, a company that is targeting NCS1 for therapeutic purposes.
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