Disease circuit verification
Figure 2. DNA sequencing chromatogram illustrating the two heterozygous mutations found in Def-HEP GSD1a.
Detection of Glycogen Storage via PAS staining
Period acid-Schiff (PAS) staining revealed Def-HEP GSD1a hepatocytes accumulated substantially greater amounts of intracellular glycogen compared with iPSC derived hepatocytes from control subjects n=3 (figure 3a); BODIPY staining showed excessive production of intracellular lipid in Def-HEP GSD1a hepatocytes (figure 3b); and Def-HEP GSD1a hepatocytes secrete more lactate compared with iPSC derived hepatocytes from control subjects, as assessed by ELISA analysis of a 24-hour collection of cell culture medium. Error bars denote SEM. N=3 (Figure 3C).
Figure 3. a) Period acid-Schiff (PAS) staining revealed Def-HEP GSD1a hepatocytes accumulated substantially greater amounts of intracellular glycogen than did those of controls (figure 3a) and showed excessive production of lipid (figure 3b) and lactic acid (figure 3c) confirming the cellular disease phenotype. (Courtesy; Rashid et. al 2010).
Figure 4. PAS/Diastase staining of cryopreserved Def-HEP GSD1a. Magnification level: x400. (A) PAS/Diastase staining showing breakdown of accumulated glycogen in the cells. (B) PAS staining showing accumulation of glycogen in the cells.
Brochure: Definigen GSD1a hepatocytes
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