Pulmonary fibrosis is a deadly disease\u00a0where scar tissue\u00a0grows\u00a0in the lungs, making breathing more difficult.\u00a0Approximately 2,170 Australians are diagnosed\u00a0annually\u00a0with idiopathic pulmonary fibrosis (IPF),\u00a0a form of the disease\u00a0with no\u00a0known cause and very few treatments.\u00a0<\/p>\n
“In pulmonary fibrosis,\u00a0the normal wound healing process in the body goes wrong. Instead of repairing damaged tissue, it starts to produce scar tissue in\u00a0the lungs,” said Associate Professor Gang Liu from the University of Technology Sydney (UTS) School of Life Sciences.\u00a0<\/p>\n
“People with\u00a0idiopathic pulmonary fibrosis\u00a0have\u00a0a very short\u00a0survival time, usually only two to five years from diagnosis.\u00a0Only two drugs are approved to treat it, and neither of them can\u00a0reverse\u00a0the scarring\u00a0and cure the disease.”\u00a0<\/p>\n
Associate Professor Liu is the co-first author on new study\u00a0published in\u00a0Science Advances\u00a0that has\u00a0identified\u00a0a protein in the lungs \u2013 vitronectin\u00a0\u2013\u00a0that can\u00a0switch on\u00a0the\u00a0scarring that\u00a0causes\u00a0pulmonary fibrosis.\u00a0<\/p>\n
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There’s\u00a0an important type of immune cell\u00a0\u2013 known as a macrophage \u2013 that helps\u00a0repair tissue after an injury. We discovered that\u00a0these macrophages sometimes get reprogrammed\u00a0to produce scarring rather than normal wound healing.”\u00a0<\/p>\n
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Gang Liu, Associate Professor, University of Technology Sydney (UTS) School of Life Sciences<\/p>\n
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Senior author\u00a0on the paper,\u00a0Associate Professor Katrina Binger from the Department of Biochemistry and Molecular Biology at Monash University,\u00a0created a 3D tissue culture system\u00a0that mimics the\u00a0fibrotic environment and\u00a0identified\u00a0vitronectin as being critical in switching the macrophages to cause scarring rather than healing.\u00a0<\/p>\n
“We normally think of vitronectin as a structural protein that\u00a0maintains\u00a0the integrity of organs like the lungs.\u00a0But we found\u00a0it also\u00a0can\u00a0also\u00a0act as a signal,” she said.\u00a0<\/p>\n
“Vitronectin\u00a0changes\u00a0how macrophages produce energy, and this drives them to have a heightened fibrotic state.\u00a0This is\u00a0a\u00a0completely\u00a0new mechanism to understand\u00a0how\u00a0fibrosis\u00a0happens that was only possible by studying these cells in more natural, 3D environments.”<\/p>\n
“Importantly, what we saw in our 3D cultures was perfectly mirrored by animal models run by Associate Professor Liu’s team and in tissue samples from patients with IPF.”<\/p>\n
The next step for the team is to identify new treatments that can use the vitronectin-macrophage pathway to treat IPF.\u00a0“Understanding this\u00a0mechanism is\u00a0critical\u00a0to\u00a0identifying\u00a0new therapeutic agents for fibrosis patients,” Associate Professor Liu said.\u00a0<\/p>\n
“Now we can work to identify new drugs that can\u00a0most\u00a0effectively inhibit vitronectin\u00a0so we can translate this research into clinical practice and help find a new cure for this\u00a0debilitating disease.”<\/p>\n
Source:<\/p>\n
University of Technology Sydney<\/a><\/p>\n Journal reference:<\/p>\n