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. 2016 May 19;7:11534. doi: 10.1038/ncomms11534

Figure 4. Involvement of a Sorbinil-sensitive aldose reductase and the kinase FGGY in the formation of CDP-ribitol.

Figure 4

(a) Potential pathways of D-ribitol-5-P synthesis from common cellular metabolites. (b) CDP-ribitol levels in ISPD-overexpressing HEK293 cells incubated without or with 3 mM D-ribose or ribitol, without or with 100 μM Sorbinil, an aldose reductase inhibitor. (c) CDP-ribitol levels in the skeletal muscle of mice treated (n=3) or not (n=4) with Sorbinil for 6 days (means±s.e.m., asterisk indicating P<0.05 in Student's t-test). Note that the ‘CDP-ribitol' peak also comprises some CDP-glucose, as shown in the MS analysis in Fig. 2. (d) Effect of ribose and ribitol on the ‘CDP-ribitol' level in ISPD-overexpressing HEK293 cells incubated in the presence of 2 different shRNAs targeting FGGY or a control shRNA. (e) α-dystroglycan glycosylation in three different HAP1 cell line clones carrying CRISPR-Cas9 double-nickase-induced mutations in FGGY, as determined by flow cytometry using the IIH6 antibody. b,d show means±s.d. (n=3), and asterisks indicate P<0.05 in Student's t-test. In all panels, the area of the CDP-ribitol peak was normalized to the total area of all peaks observed at 280 nm (a.u., arbitrary units).