The other 2 ALG5 missense substitutions in ADPKD families (p.R208 and p.R212) are similar to p.R79,6 in which the variant also replaces an arginine residue in the close proximity of the A-loop of the enzyme that governs dolichyl-phosphate beta-glucosyltransferase activity (Figure 3b and c). This evidence concerns the gene ALG5 and autosomal dominant polycystic kidney disease.