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. 2017 Apr;137(4):845–854. doi: 10.1016/j.jid.2016.12.010

Figure 3.

Figure 3

VPS33B(p.Gly131Glu) cannot rescue defective LH3 delivery in VPS33B-deficient mIMCD3 cells. (a–b) Representative, (a) Control, or (b) VPS33B shRNA treated mIMCD3 cells expressing LH3-mCherry, stained for collagen IV (c–d) Representative VPS33B shRNA-treated mIMCD3 cells expressing (c) YFP-VPS33B or (d) YFP-VPS33B(p.Gly131Glu) and CFP-VIPAR, LH3-mCherry and stained for collagen IV. (e) Quantification of LH3-mCherry/collagen IV colocalization. One-way ANOVA, **P ≤ 0.01, ****P ≤ 0.0001. (f, g) LC-MS-MS analysis for relative quantification of hydroxylysines (Lys-OH), galactosyl-hydroxylysines (Lys-O-Gal), and glucosylgalactosyl-hydroxylysines (Lys-O-GalGlc) from (f) urine of patient 1 and father, patient 2 and mother, age-matched controls, and patients with ARC, (g) collagen I from control, patient 2, and ARC patient skin fibroblasts, statistics not performed as only single patient samples were analyzed. Scale bars = 10 μm. Data are mean ± standard deviation. ANOVA, analysis of variance; ARC, arthrogryposis renal dysfunction and cholestasis; CFP, cyan fluorescent protein; LC-MS-MS, liquid chromatography tandem mass spectrometry; mIMCD3, murine inner medullary collecting duct 3; wt, wild type; YFP, yellow fluorescent protein.