Augmented NRF2 activation protects adult sickle mice from lethal acute chest syndrome.
Br J Haematol
; 182(2): 271-275, 2018 07.
Article
en En
| MEDLINE
| ID: mdl-29923176
ABSTRACT
Acute chest syndrome (ACS) mortality in sickle cell disease (SCD) rises sharply in young adult patients and mechanism-based prophylaxis is lacking. In SCD, haem oxygenase-1 (HO-1) declines with age and ACS is associated with low HO-1. To test if enhanced HO-1 can reduce ACS mortality, young SCD mice were treated with D3T (3H-1,2-dithiole-3-thione), an activator of nuclear-factor erythroid 2 like 2, which controls HO-1 expression, for 3 months. Following haem-induced ACS, all vehicle-treated mice succumbed to severe lung injury, while D3T-treated mice had significantly improved survival. Blocking HO-1 activity abrogated the D3T effect. Thus HO-1 may be targeted to reduce ACS severity in adult patients.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Factor 2 Relacionado con NF-E2
/
Síndrome Torácico Agudo
Límite:
Animals
Idioma:
En
Revista:
Br J Haematol
Año:
2018
Tipo del documento:
Article
País de afiliación:
Estados Unidos