Details for phenotype: pulmonary fibrosis

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EndoNet ID: ENP00333

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Name

pulmonary fibrosis

General information

This phenotype is pathologic

Links to other resources

GO	regulation of tissue remodeling
OMIM	178500
Medline Plus	000069
MeSH term	D011658
Disease database	4815

Phenotype triggers

• more activity (high ligand concentration, overexpression) of Lysophosphatidic acid receptor 1 ui-button
  in lung ui-button
  • Fibrosis, the formation of excess fibrous connective tissues, is associated with a number of pathological conditions. Recently, a new aspect of LPA1 signaling has been uncovered in pulmonary fibrosis, suggesting LPA1 signaling as a new therapeutic target in this disease. LPA levels were remarkably increased in bronchoalveolar lavage fluid after bleomycin-induced lung injury and resulted in pulmonary fibrosis, vascular leakage, and mortality. These pathologies were markedly reduced in Lpar1-deficient mice. [1]

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