DISTRIBUTION OF LYSOSOMAL PROTECTIVE PROTEIN IN HUMAN TISSUES

We raised two polyclonal antibodies against synthetic oligopeptides co mprising amino acid sequences in the human lysosomal protective protei n. The first antibody recognized the 54-kDa precursor and the N-termin al sequence of the 32-kDa mature protein subunit, and the second one r ecognized the precursor and the C-terminal sequence of the 20-kDa subu nit. In normal fibroblasts, mature protective protein was detected on immunoblotting with these antibodies. Considerable amounts of mature p rotective protein also were detected in kidney, lung, liver, and splee n, but not in brain from a patient with Gaucher disease. Neither the p recursor nor the mature protective protein was detected in cultured fi broblasts, liver or cerebrum from a galactosialidosis patient with pro tective protein deficiency. (C) 1994 Academic Press, Inc.