RT Journal Article
T1 Toll‑like receptors ligand immunomodulators for the treatment congenital diaphragmatic hernia.
A1 Vallejo-Cremades, Mayte
A1 Merino, Javier
A1 Carmona-Mejías, Rita María
A1 Córdoba, Laura
A1 Salvador, Beatriz
A1 Martínez, Leopoldo
A1 Tovar, Juan Antonio
A1 Llamas, Miguel Ángel
A1 Muñoz-Chápuli-Oriol, Ramón
A1 Fresno, Manuel
K1 Cardiopatía congénita
K1 Corazón - Embriología
K1 Genética del desarrollo
K1 Macrófagos
K1 Interacción celular
AB Congenital diaphragmatic hernia (CDH) is a rare disease that affects the development of the diaphragm,leading to abnormal lung development. Unfortunately, there is no established therapy for CDH. Retinoicacid pathways are implicated in the ethology of CDH and macrophages are known to play a role in repairing organdamage. We have analyzed the effect of several Toll like receptor (TLR) ligands in the nitrofen-induced CDH modelin pregnant rats widely used to study this disease and in the G2-GATA4Cre;Wt1fl/fl CDH genetic mice model. Morphometricand histological studies were carried out. Immune cell infiltration was assayed by immunochemistryand immunofluorescence and retinoic pathway gene expression analyzed in vivo and in vitro in macrophages. Our research has shown that TLR ligand immunomodulators that influence anti-inflammatory macrophageactivation can be effective in treating CDH, being nontoxic for the mothers or pups suggesting that thoseTLR ligands are a promising solution for CDH leading to orphan drug designation for CS1. The immune systemof the fetus would be responsible for repairing the damage and closure of the hernia in the diaphragm and enhancedproper lung development after CS1 treatment.
PB BMC
YR 2024
FD 2024
LK https://hdl.handle.net/10630/38984
UL https://hdl.handle.net/10630/38984
LA eng
NO Vallejo‑Cremades et al. Orphanet Journal of Rare Diseases (2024) 19:386 https://doi.org/10.1186/s13023-024-03384-7
DS RIUMA. Repositorio Institucional de la Universidad de Málaga
RD 20 ene 2026