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Figure 7 | Orphanet Journal of Rare Diseases

Figure 7

From: In Lysinuric Protein Intolerance system y+L activity is defective in monocytes and in GM-CSF-differentiated macrophages

Figure 7

Effect of GM-CSF on System y+L activity and transporters expression. Normal and LPI monocytes were differentiated to Monocyte-Derived Marcophages (MDM) as described in Figure 6. Panel A. The activities of system y+L and system y+ were determined as described in Figure 3, both in freshly isolated (control) and in rGM-CSF-treated (MDM) monocytes. For normal cells, data are means ± SEM of 6 independent experiments (n = 6 normal donors). For the LPI patient, data are means ± SD of 4 determinations in a representative experiment that was repeated twice with comparable results. ***p < 0.001 MDM vs control. Panel B. In the same conditions SLC7A6 and SLC7A7 expression were determined as numbers of molecules of mRNA indexed to RPL mRNA (see Methods). For normal cells, data are means ± SEM of 6 independent experiments (n = 6 normal donors). For the LPI patient, data are means ± SD of 4 determinations. * p < 0.05 MDM vs control.

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