The invention relates to .alpha.-galactosidase truncated at the carboxy
terminus and the production of enzymatically active recombinant human and
animal lysosomal enzymes involving construction and expression of
recombinant expression constructs comprising coding sequences of human or
animal lysosomal enzymes in a plant expression system. The plant
expression system provides for post-translational modification and
processing to produce a recombinant gene product exhibiting enzymatic
activity. The invention is demonstrated by working examples in which
transgenic tobacco plants express recombinant expression constructs
comprising human glucocerebrosidase nucleotide sequences. The invention
is also demonstrated by working examples in which transfected tobacco
plants express recombinant viral expression constructs comprising human
.alpha. galactosidase nucleotide sequences. The recombinant lysosomal
enzymes produced in accordance with the invention may be used for a
variety of purposes, including but not limited to enzyme replacement
therapy for the therapeutic treatment of human and animal lysosomal
storage diseases.
