Key cell and protein players in the genetic lung disease pulmonary alveolar microlithiasis revealed
Every breath can be a struggle if you have the rare lung disease pulmonary alveolar microlithiasis. It’s caused by a faulty protein in the membranes of cells that line alveoli, tiny air sacs that fill your lungs. This causes mineral structures called hydroxyapatite microliths to form inside alveoli. Researchers now investigate this process by analysing RNA – a marker for gene activity – in lung tissue from human patients and a mouse model of the disease. Looking at immune cells called monocytes, which can develop into bone-degrading cells called osteoclasts, the team found that osteoclast-related genes were activated in alveolar monocytes. They also found that microliths, pictured using scanning electron microscopy at different resolutions in human (top) and mouse (bottom) lung tissue, contained osteoclast enzymes. This suggests that in response to microliths forming, osteoclast-like cells kickstart into action. This may present a new research avenue for pulmonary alveolar microlithiasis therapies.
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