Compromised Cardiopulmonary Function in Fibulin-5 Deficient Mice.

ABSTRACT: Competent elastic fibers are critical to the function of the lung and right circulation. Murine models of elastopathies can aid in understanding the functional roles of the elastin and elastin-associated glycoproteins that constitute elastic fibers. Here, we quantify together lung and pulmonary arterial structure, function, and mechanics with right heart function in a mouse model deficient in the elastin-associated glycoprotein fibulin-5. Differences emerged as a function of genotype, sex, and arterial region. Specifically, functional studies revealed increased lung compliance in fibulin-5 deficiency consistent with a histologically observed increased alveolar disruption. Biaxial mechanical tests revealed that the primary branch pulmonary arteries exhibit decreased elastic energy storage c

SUBMITTER: Ramachandra AB 

PROVIDER: S-EPMC8990734 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature