A case report on filamin A gene mutation and progressive pulmonary disease in an infant: A lung tissued derived mesenchymal stem cell study
Academic Article
Publication Date:
2018
abstract:
Mesenchymal stem cells (MSC) play a crucial role in both the maintenance of pulmonary integrity and the pathogenesis of lung disease. Lung involvement has been reported in patients with the filamin A (FLNA) gene mutation. Considering FLNA's role in the intrinsic mechanical properties of MSC, we characterized MSCs isolated from FLNA-defective lung tissue, in order to define their pathogenetic role in pulmonary damage.
Iris type:
1.1 Articolo in rivista
Keywords:
Biopsy; Cell Differentiation; Filamins; Humans; Infant; Italy; Lung; Lung Diseases; Male; Mesenchymal Stem Cells
List of contributors:
Calcaterra, Valeria; Avanzini, Maria Antonietta; Mantelli, Melissa; Agolini, Emanuele; Croce, Stefania; De Silvestri, Annalisa; Re, Giuseppe; Collura, Mirella; Maltese, Alice; Novelli, Antonio; Pelizzo, Gloria
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