Fig. 1: Mutations in AGL cause glycogen buildup and glucose-induced embryonic lethality in C. elegans models of GSDIII. | Communications Biology

Fig. 1: Mutations in AGL cause glycogen buildup and glucose-induced embryonic lethality in C. elegans models of GSDIII.

From: CHK1 inhibition rescues abnormal glycogen buildup in a Caenorhabditis elegans model for glycogen storage disease III

Fig. 1: Mutations in AGL cause glycogen buildup and glucose-induced embryonic lethality in C. elegans models of GSDIII.

A Location of the AGL mutations in the glucosidase domain. B Evolutionary conservation of affected residue in C. elegans AGL-1 ortholog (highlighted in yellow). Protein alignment was done using MARVEL. C Imaging protocol for iodine staining from bright field to image processing. From left to right: schematic of 3D printed pad used for staining, an example of an acquired image after staining, and image processing using a macro in Fiji software for quantification. Control and test worms are exposed to iodine simultaneously. D Representative image of glycogen buildup visualized by iodine staining in an agl-1 mutant compared to wild-type. E Iodine staining quantification shows significantly increasing glycogen buildup in agl-1 mutants compared to wild-type at day 1 of adulthood (n ≈ 30). F Glycogen quantification using a calorimetric kit shows significantly increased levels of glycogen in mutants compared to wild-type at day 1 of adulthood (N = 3). G and H Embryonic viability assay is significantly decreased in mutants when exposed to 2% of glucose, with no significant differences in brood size (n ≈ 30). One-way ANOVA was performed for E (DF = 3; F(3,97) = 1.380), F (DF = 3; F(3,41) = 1.058), G (DF = 3; F(3,350) = 18.85) and H (DF = 3; F(3,8) = 1.513). Data are presented as mean ± SEM.

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