title: Partial absence of pleuropericardial membranes in Tbx18- and Wt1-deficient mice.
abstract: The pleuropericardial membranes are fibro-serous walls that separate the pericardial and pleural cavities and anchor the heart inside the mediastinum. Partial or complete absence of pleuropericardial membranes is a rare human disease, the etiology of which is poorly understood. As an attempt to better understand these defects, we wished to analyze the cellular and molecular mechanisms directing the separation of pericardial and pleural cavities by pleuropericardial membranes in the mouse. We found by histological analyses that both in Tbx18- and Wt1-deficient mice the pleural and pericardial cavities communicate due to a partial absence of the pleuropericardial membranes in the hilus region. We trace these defects to a persisting embryonic connection between these cavities, the pericardioperitoneal canals. Furthermore, we identify mesenchymal ridges in the sinus venosus region that tether the growing pleuropericardial membranes to the hilus of the lung, and thus, close the pericardioperitoneal canals. In Tbx18-deficient embryos these mesenchymal ridges are not established, whereas in Wt1-deficient embryos the final fusion process between these tissues and the body wall does not occur. We suggest that this fusion is an active rather than a passive process, and discuss the interrelation between closure of the pericardioperitoneal canals, lateral release of the pleuropericardial membranes from the lateral body wall, and sinus horn development.
authors.affiliation: Institut für Molekularbiologie, OE5250, Medizinische Hochschule Hannover, Hannover, Germany.
authors.forename: Julia
authors.initials: J
authors.lastname: Norden
authors.forename: Thomas
authors.initials: T
authors.lastname: Grieskamp
authors.forename: Vincent M
authors.initials: VM
authors.lastname: Christoffels
authors.forename: Antoon F M
authors.initials: AF
authors.lastname: Moorman
authors.forename: Andreas
authors.initials: A
authors.lastname: Kispert
mesh_terms: D000818:Animals; D049109:Cell Proliferation; D004622:Embryo, Mammalian / Q000196:embryology / Q000378:metabolism; D005260:Female; D018507:Gene Expression Regulation, Developmental; D049452:Green Fluorescent Proteins / Q000235:genetics / Q000378:metabolism; D006321:Heart / Q000196:embryology*; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008297:Male; D051379:Mice; D008815:Mice, Inbred Strains; D018345:Mice, Knockout; D008822:Mice, Transgenic; D008856:Microscopy, Fluorescence; D010496:Pericardium / Q000002:abnormalities* / Q000378:metabolism; D011247:Pregnancy; D020825:T-Box Domain Proteins / Q000172:deficiency* / Q000235:genetics; D013997:Time Factors; D025721:WT1 Proteins / Q000172:deficiency* / Q000235:genetics
chemical_list: D020825:T-Box Domain Proteins; C420983:Tbx18 protein, mouse; D025721:WT1 Proteins; D049452:Green Fluorescent Proteins