MPDU1 mutations underlie a novel human congenital disorder of glycosylation, designated type If
J. Clin. Invest. Barbara Schenk, et al. 108:1687 doi:10.1172/JCI13419 [
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Figure 2Analysis of NLOs in CDG patients and a control subject. Metabolically labeled NLOs were isolated from a healthy individual (control subject) and from patients A, L, and S (CDG-If patient A, CDG-If patient L, CDG-If patient S). [
3H]-labeled NLOs were cleaved from protein by incubation with PNGaseF, protein was removed by precipitation, and the liberated oligosaccharides were analyzed by HPLC using a radiodetector to monitor elution positions. Elution positions for standard oligosaccharide intermediates Man
3GlcNAc
2 to Glc
1Man
9GlcNAc
2 (M
3, M
4, M
5, M
6, M
7, M
8, M
9, G
1M
9) are given at the top of the figure.