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Down‐regulation of dysferlin and myoferlin in human airway epithelium: differential effects on cell morphology and adhesion
Author(s) -
Leung Cleo,
Shaheen Furquan,
Hackett Tillie,
Bernatchez Pascal
Publication year - 2012
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.26.1_supplement.697.3
Subject(s) - dysferlin , microbiology and biotechnology , gene knockdown , respiratory epithelium , biology , epithelium , cell culture , muscular dystrophy , genetics
Dysferlin and myoferlin belong to Ferlin protein family known to mediate endomembrane fusion events. Mutations of dysferlin lead to muscular dystrophy. Patients with dysferlinopathy show cardiopulmonary complications, suggesting that dysferlin may have functional roles in the pulmonary system. The high homology of myoferlin to dysferlin proposes that myoferlin may also play a role in lung pathology. Objectives To examine dysferlin and myoferlin expression in human lungs and investigate the effects of silencing these genes in human airway epithelial cells (HAEC). Methods Dysferlin and myoferlin expression was evaluated by immunohistochemistry in human airway sections and by Western blot analysis in HAEC. Localization of dysfelrin and myoferlin in HAEC was studied using immunofluorescent staining. Effects of dysferlin and myoferlin knockdown on the morphology and adhesion of HAEC were also assessed. Results Both dysferlin and myoferlin were present in the epithelium of human airway tissues and cultured HAEC. Dysferlin and myoferlin expression localized at the membrane and cytoplasm of HAEC. Knockdown of myoferlin, but not dysferlin, resulted in cell morphological changes, deficient cell adhesion and decreased the expression of zonular occludens 1 in HAEC. Conclusion Presence of dysferlin and myoferlin suggests they play potential roles in the adhesion of human airway epithelial cells.