The fate of internalized alpha-2-macroglobulin: alpha-galactosidase conjugate in fibroblasts from Fabry's hemizygote.
In our previous report we described the endocytotic incorporation of coffee bean alpha-galactosidase conjugated to human alpha-2-macroglobulin (alpha-2-M) into cultured fibroblasts derived from a patient with Fabry's disease (1). The fate of internalized alpha-galactosidase according to the method described in the above report is now studied. Measurement of the enzyme activity of subcellular fractions showed that it was concentrated in the lysosomal-mitochondrial fraction. The half-life of internalized alpha-galactosidase was determined to be 2 h.
D002478:Cells, Cultured; D000795:Fabry Disease / Q000188:drug therapy / Q000378:metabolism*; D005347:Fibroblasts / Q000378:metabolism; D005696:Galactosidases / Q000378:metabolism*; D006207:Half-Life; D006801:Humans; D026503:Low Density Lipoprotein Receptor-Related Protein-1; D011971:Receptors, Immunologic / Q000378:metabolism; D013347:Subcellular Fractions / Q000378:metabolism; D000519:alpha-Galactosidase / Q000378:metabolism* / Q000627:therapeutic use; D000511:alpha-Macroglobulins / Q000008:administration & dosage / Q000378:metabolism*
D026503:Low Density Lipoprotein Receptor-Related Protein-1; D011971:Receptors, Immunologic; D000511:alpha-Macroglobulins; D005696:Galactosidases; D000519:alpha-Galactosidase