RT Journal Article
T1 Bicuspid Aortic Valve in 2 Model Species and Review of the Literature
A1 Fernández-Corujo, Borja
A1 Soto-Navarrete, María Teresa
A1 López-García, Alejandro
A1 López-Unzu, Miguel A.
A1 Durán-Boyero, Ana Carmen
A1 Fernández-Domínguez, María Carmen
K1 Válvula aórtica
K1 Cardiopatía congénita
K1 Enfermedades - Etiología
K1 Ratones
K1 Hámsteres
AB Bicuspid aortic valve (BAV) is the most common human congenital cardiac malformation. Although the etiology is unknown formost patients, formation of the 2 main BAV anatomic types (A and B) has been shown to rely on distinct morphogenetic mechanisms. Animal models of BAV include 2 spontaneous hamster strains and 27 genetically modified mouse strains. To assessthe value of these models for extrapolation to humans, we examined the aortic valve anatomy of 4340 hamsters and 1823 micefrom 8 and 7 unmodified strains, respectively. In addition, we reviewed the literature describing BAV in nonhuman mammals. Theincidences of BAV types A and B were 2.3% and 0.03% in control hamsters and 0% and 0.3% in control mice, respectively.Hamsters from the spontaneous model had BAV type A only, whereas mice from 2 of 27 genetically modified strains had BAV typeA, 23 of 27 had BAV type B, and 2 of 27 had both BAV types. In both species, BAV incidence was dependent on genetic background. Unlike mice, hamsters had a wide spectrum of aortic valve morphologies. We showed interspecific differences in theoccurrence of BAV between humans, hamsters, and mice that should be considered when studying aortic valve disease usinganimal models. Our results suggest that genetic modifiers play a significant role in both the morphology and incidence of BAV. Wepropose that mutations causing anomalies in specific cardiac morphogenetic processes or cell lineages may lead to BAV types A, B, or both, depending on additional genetic, environmental, and epigenetic factors
PB Sage Publications INC
YR 2020
FD 2020-02-21
LK https://hdl.handle.net/10630/30007
UL https://hdl.handle.net/10630/30007
LA spa
NO Fernández B, Soto-Navarrete MT, López-García A, López-Unzu MÁ, Durán AC, Fernández MC. Bicuspid Aortic Valve in 2 Model Species and Review of the Literature. Veterinary Pathology. 2020;57(2):321-331.
NO Junta de Andalucía (grant P10-CTS-6068).Ministerio de Ciencia, Innovación y Universidades (grant CGL2017-85090-P and fellowships FPU15/03209 to M.A.L.-U. and PRE2018-083176 to M.T.S.-N.).Sistema Nacional de Garantía Juvenil (contract UMAJI75 to M.T.S.-N.).FEDER funds.
DS RIUMA. Repositorio Institucional de la Universidad de Málaga
RD 21 ene 2026