Contribution of collagen XIII to lung function and development of pulmonary fibrosis

CONCLUSIONS: Changes in mouse lung function appear to represent a myasthenic manifestation of collagen XIII deficiency. We suggest that respiratory muscle myasthenia is the primary cause of the breathing problems suffered by CMS19 patients in addition to skeletal deformities. Induction of collagen XIII expression in the IPF patients' lungs warrants further studies to reveal collagen XIII-dependent disease mechanisms.PMID:38568728 | DOI:10.1136/bmjresp-2023-001850

Source: Cell Research - April 3, 2024 Category: Cytology Authors: Oula Norman Jarkko Koivunen Riitta Kaarteenaho Antti M Salo Joni M M äki Johanna Myllyharju Taina Pihlajaniemi Anne Heikkinen Source Type: research