Fig. 1. Sulf1^-/-;Sulf2^-/- mice have cellular HS 6-O-sulfation, postnatal growth and dysfunctional esophageal phenotypes. (A) Disaccharide analysis of HS. Radiolabeled HS was isolated from MEFs of wild-type, Sulf1^-/-, Sulf2^-/- and Sulf1^-/-;Sulf2^-/- embryos at E14.5, followed by disaccharide analysis. Individual disaccharides are represented as the percentage of the total radioactivity. Data presented are mean and standard deviation of a minimum of two independent samples of each genotype. In disaccharide abbreviation, M stands for the 2,5-anhydromannitol deamination products of GlcNS residues. ^**, P<0.01; ^*, P<0.05. (B) Comparison of the body size and weight between wild-type, Sulf1^-/-, Sulf2^-/- and Sulf1^-/-;Sulf2^-/- female mice at P12 and after weaning (n=6 for each group). (C) Histology of the adult esophagus and lung of wild-type control and Sulf1^-/-;Sulf2^-/- mice. Sulf1^-/-;Sulf2^-/- mice have enlarged esophagi with food accumulated inside (compare a1,a2 with b1,b2, respectively) and develop lung infections (n=14). Eso, esophagus; ME, muscularis externa; MM, muscularis mucosae. Scale bars: 100 µm.