Publication: Mast cell chymase: an indispensable
instrument in the pathological symphony
of idiopathic pulmonary fibrosis?
Authors
Kosanovic, Djuro ; Daha, Bhola Kumar ; Wygrecka, Malgorzata ; Reiss, Irwin ; Günther, Andreas ; Ghofrani, Ardeschir ; Weissmann, , Norbert ; Grimminger, Friedrich ; Seeger, Werner ; Schermuly, Ralph Theo ; Banat, Gamal-Andre
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Publisher
F. Hernández y Juan F. Madrid. Universidad de Murcia: Departamento de Biología Celular e Histología
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Idiopathic pulmonary fibrosis (IPF) is a
chronic, progressive and fatal lung disease with no
known etiology and treatment options. The hallmarks of
the histopathology, which is characteristic of usual
interstitial pneumonia (UIP) pattern, include interstitial
fibrosis, honeycomb changes and fibroblast foci that
develop owing to fibroblast proliferation and excessive
matrix deposition. Although the complete pathomechanism
is not yet understood, several molecular
culprits, including transforming growth factor (TGF)-ß,
Angiotensin (Ang) II, endothelin (ET)-1, matrix
metalloproteinases (MMPs) and cytokines have been
identified. IPF is increasingly believed to be an
epithelial-driven disease; however, the literature does
support an implication of altered immune response and
inflammatory processes in the onset or progression of
the disease. Mast cells (MCs) are multifunctional tissue
resident cells involved in the inflammatory and immune
response. An increasing body of evidence suggests a role
of MCs and their mediator chymase in the pathology of
IPF. With regard to the underlying mechanisms, it is
conceivable that MC chymase may function via
activation or processing of factors such as proteases,
cytokines and growth factors. In this review, we will
discuss how MC chymase is linked to and can
potentially contribute to the development of IPF.
Moreover, the findings from animal model studies will
be discussed to highlight the chymase inhibitors as a
promising strategy for the treatment of pulmonary
fibrosis.
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Citation
Histology and Histopathology, vol. 28, nº 6, (2013)
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