Fig. 1. (A) Generation of LAP/Acp5 doubly deficient mice. Southern blot with external probes for the targeted LAP locus (Saftig et al., 1997) and the Acp5 locus (Hayman et al., 1996). (B) Determination of lysosomal acid phosphatase activity (Waheed et al., 1985) in enriched lysosomal liver fractions of control, single deficient and doubly deficient mice. Acp5 activity was determined after immunoprecipitation with an anti-uteroferrin antibody. (C) LAP/Acp5 doubly knockout mice display an increased mortality starting at about 8 months of age. The results from LAP and Acp5 single knockout as well as control animals are also shown. (D) Hepatosplenomegaly in LAP/Acp5 deficient animal. The spleen and liver of an extreme case of hepatosplenomegaly in LAP/Acp5 knockout animals is shown. (E) Progressive increase in liver weight in LAP/Acp5-deficient mice. A box blot of control (striped boxes) and LAP/Acp5 deficient liver weights is shown from 1-15 month of age. (F) Progressive increase in spleen weight in LAP/Acp5-deficient mice. A box plot of control (striped boxes) and LAP/Acp5 deficient spleen weights is shown from 1-15 month of age.
