Deficiency of WFS1 leads to the impairment of AVP secretion under dehydration in male mice.
Arginine vasopressin
BiP
Diabetes insipidus
Endoplasmic reticulum stress
Wolfram syndrome
Journal
Pituitary
ISSN: 1573-7403
Titre abrégé: Pituitary
Pays: United States
ID NLM: 9814578
Informations de publication
Date de publication:
Aug 2021
Aug 2021
Historique:
accepted:
08
02
2021
pubmed:
6
3
2021
medline:
15
12
2021
entrez:
5
3
2021
Statut:
ppublish
Résumé
Wolfram syndrome (WS) is mainly caused by mutations in the WFS1 gene and characterized by diabetes mellitus, optic atrophy, hearing loss, and central diabetes insipidus (CDI). WFS1 is an endoplasmic reticulum (ER)-resident transmembrane protein, and Wfs1 knockout (Wfs1-/-) mice, which have been used as a mouse model for WS, reportedly manifested impairment of glucose tolerance due to pancreatic β-cell loss. In the present study, we examined water balance, arginine vasopressin (AVP) secretion, and ER stress in AVP neurons of the hypothalamus in Wfs1-/- mice. There were no differences in urine volumes between Wfs1-/- and wild-type mice with free access to water. Conversely, when mice were subjected to intermittent water deprivation (WD) for 20 weeks, during which water was unavailable for 2 days a week, urine volumes were larger in Wfs1-/- mice, accompanied by lower urine AVP concentrations and urine osmolality, compared to wild-type mice. The mRNA expression of immunoglobulin heavy chain binding protein, a marker of ER stress, was significantly increased in the supraoptic nucleus and paraventricular nuclei in Wfs1-/- mice compared to wild-type mice after WD. Our results thus showed that Wfs1 knockout leads to a decrease in AVP secretion during dehydration, which could explain in part the mechanisms by which Wfs1 mutations cause CDI in humans.
Identifiants
pubmed: 33666833
doi: 10.1007/s11102-021-01135-6
pii: 10.1007/s11102-021-01135-6
doi:
Substances chimiques
Membrane Proteins
0
wolframin protein
0
Water
059QF0KO0R
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
582-588Informations de copyright
© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC part of Springer Nature.
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