Figure 4

General phenotype of transgenic ASA−/− mice (hatched bars and symbols), non-transgenic ASA−/− mice (open bars and symbols) and wildtype mice (closed bars and symbols). Bars and points indicate means ± SDs. (A) Body weight. n = 10. (B) Organ weights at age 6 mo. n = 10. (C) Littering. The litter frequency indicates the total number of litters produced by five to seven permanent breeding pairs mated at age five to nine weeks for six months. Litter sizes and lethality before age five weeks was evaluated for 33–64 litters. (D) Fraction of male pups among 83–363 newborns. (E) Activity of sulfatases in serum. n = 3. hASA-C69S serum levels of transgenic ASA−/− mice: 0.3–0.7 µg/mL. (F) Serum activities of β-hexosaminidase (β-hex), α-galactosidase (α-gal), β-galactosidase (β-gal) and lysosomal acid phosphatase (LAP). Same mice as in (E).