Diseased fibroblasts are isolated from adult lung tissue of cystic fibrosis donors. The most abundant cell type in lung interstitium is fibroblasts. They resemble ordinary fibroblasts but have some distinguishing features, for example, they have long branching processes and gap junctions. Their principle function is production of type III collagen, elastin, and proteoglycans of the extracellular matrix of the alveolar septa. Pulmonary fibroblasts (PF) play an important role in the repair and remodeling processes following injury. The controlled accumulation of fibroblasts to sites of inflammation is crucial to effective tissue repair after injury. Either inadequate or excessive accumulation of fibroblasts could result in abnormal tissue function. For example, the excess proliferation of fibroblasts contributes to the adventitial thickening observed during the development of hypoxia-induced pulmonary hypertension. Cryopreserved Diseased Lung Fibroblasts from Cystic Fibrosis donors stain negative for von Willebrand factor VIII, cytokeratins 18 and 19, and alpha smooth muscle actin. All cells test negative for mycoplasma, bacteria, yeast, and fungi. HIV-1, hepatitis B and hepatitis C are not detected for all donors and/or cell lots. A Certificate of Analysis is provided for each cell lot purchased.
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