Figure 4.

The ALG10 frameshift mutation causes defects in protein N-glycosylation due to a predicted defect in alpha-1,2-glucosyltransferase activity

(A) Affected protein glycosylation in fibroblasts carrying the ALG10 and ALG10B variants. Western blot analysis of ICAM1 and LAMP1 expression. HSP90 was used as loading control.

(B) Protein N-glycosylation of CPY shows multiple hypo-glycosylated bands in a yeast alg10 deletion strain transformed with mutated human (h) ALG10 (hALG10fs) or empty vector. N-glycosylation deficiency is rescued when transformed with either wild-type yeast ALG10 (yALG10), hALG10, or hALG10B.