Muc5b-deficient mice develop early histological lung abnormalities.

Autor:	Valque H; LIRIC UMR 995; Univ. Lille; Inserm; CHU Lille, F-59045 Lille, France., Gouyer V; LIRIC UMR 995; Univ. Lille; Inserm; CHU Lille, F-59045 Lille, France., Duez C; CIIL Inserm U1019; CNRS UMR 8204; Institut Pasteur de Lille; Univ. Lille, F-59019 Lille, France., Leboeuf C; Inserm UMR_S 1165; Université Paris- Diderot, Institut Universitaire d'Hématologie, AP-HP-Hôpital Saint Louis, F-75010 Paris, France., Marquillies P; CIIL Inserm U1019; CNRS UMR 8204; Institut Pasteur de Lille; Univ. Lille, F-59019 Lille, France., Le Bert M; CNRS UMR 7355, University of Orleans, Laboratory of Experimental and Molecular Immunology and Neurogenetics (INEM), F-45071 Orleans, France., Plet S; LIRIC UMR 995; Univ. Lille; Inserm; CHU Lille, F-59045 Lille, France., Ryffel B; CNRS UMR 7355, University of Orleans, Laboratory of Experimental and Molecular Immunology and Neurogenetics (INEM), F-45071 Orleans, France.; IDM, University of Cape Town, South Africa., Janin A; Inserm UMR_S 1165; Université Paris- Diderot, Institut Universitaire d'Hématologie, AP-HP-Hôpital Saint Louis, F-75010 Paris, France., Gottrand F; LIRIC UMR 995; Univ. Lille; Inserm; CHU Lille, F-59045 Lille, France., Desseyn JL; LIRIC UMR 995; Univ. Lille; Inserm; CHU Lille, F-59045 Lille, France jean-luc.desseyn@inserm.fr.
Jazyk:	angličtina
Zdroj:	Biology open [Biol Open] 2019 Nov 07; Vol. 8 (11). Date of Electronic Publication: 2019 Nov 07.
DOI:	10.1242/bio.046359
Abstrakt:	Gel-forming mucins are the main organic component responsible for physical properties of the mucus hydrogels. While numerous biological functions of these mucins are well documented, specific physiological functions of each mucin are largely unknown. To investigate in vivo functions of the gel-forming mucin Muc5b, which is one of the major secreted airway mucins, along with Muc5ac, we generated mice in which Muc5b was disrupted and maintained in the absence of environmental stress. Adult Muc5b-deficient mice displayed bronchial hyperplasia and metaplasia, interstitial thickening, alveolar collapse, immune cell infiltrates, fragmented and disorganized elastin fibers and collagen deposits that were, for approximately one-fifth of the mice, associated with altered pulmonary function leading to respiratory failure. These lung abnormalities start early in life, as demonstrated in one-quarter of 2-day-old Muc5b-deficient pups. Thus, the mouse mucin Muc5b is essential for maintaining normal lung function. Competing Interests: Competing interestsThe authors declare no competing or financial interests. (© 2019. Published by The Company of Biologists Ltd.)
Databáze:	MEDLINE
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