Deficiency of Circulating Monocytes Ameliorates the Progression of Myxomatous Valve Degeneration in Marfan Syndrome.

ABSTRACT:

Background

Myxomatous valve degeneration (MVD) involves the progressive thickening and degeneration of the heart valves, leading to valve prolapse, regurgitant blood flow, and impaired cardiac function. Leukocytes composed primarily of macrophages have recently been detected in myxomatous valves, but the timing of the presence and the contributions of these cells in MVD progression are not known.

Methods

We examined MVD progression, macrophages, and the valve microenvironment in the context of Marfan syndrome (MFS) using mitral valves from MFS mice (Fbn1^C1039G/+), gene-edited MFS pigs (FBN1^{Glu433AsnfsX98/+}), and patients with MFS. Additional histological and transcriptomic evaluation was performed by using nonsyndromic human

SUBMITTER: Kim AJ 

PROVIDER: S-EPMC7017394 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature