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Chunk #26 — DISCUSSION

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Musculocontractural Ehlers-Danlos syndrome and neurocristopathies: dermatan sulfate is required for Xenopus neural crest cells to migrate and adhere to fibronectin.
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A human esophagus squamous cell carcinoma cell line with short hairpin RNA (shRNA)-mediated DS-epi1 loss-of-function (Thelin et al., 2012) and cultured aortic smooth muscle cells from Dse−/− mice (Bartolini et al., 2013) both have a reduced level of plasma membrane protrusions and an abnormal actin cytoskeleton, which changes their migration properties similar to the findings in CNC cells. Interestingly, DS-epi1 deficiency in these in vitro wound scratch and invasion assays leads to a larger cell size and increased speed, but decreased directionality, of cell migration. In comparison, DS-epi1 morphant CNC cells had a smaller cell size and did not migrate in vitro because of a failure in cell adhesion to fibronectin. These discrepancies might be a result of the different cell types and matrix components.