Transport of radiolabelled glycoprotein to cell surface and lysosome-like bodies of absorptive cells in clutured small-intestinal tissue from normal subjects and patients with a lysosomal storage disease.
The transport of 3H-fucose- and 3H-glucosamine-labelled glycoproteins in the absorptive cells of cultured human small-intestinal tissue was investigated with light- and electron-microscopical autoradiography. The findings showed that these glycoproteins were completed in the Golgi apparatus and transported in small vesicular structures to the apical cytoplasm of these cells. Since this material arrived in the cell coat on the microvilli and in the lysosome-like bodies simultaneously, a crinophagic function of these organelles in the regulation of the transport or secretion of cell-coat material was supported. In the absorptive cells of patients with fucosidosis or Hunter's type of lysosomal storage disease, a smiliar transport of cell-coat material to the lysosome-like bodies and a congenital defect of a lysosomal hydrolase normally involved in the degradation of cell-coat material, can explain the accumulation of this material in the dense bodies.
D000293:Adolescent; D000328:Adult; D001345:Autoradiography; D001692:Biological Transport; D002462:Cell Membrane / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D046508:Culture Techniques; D005260:Female; D005643:Fucose / Q000378:metabolism*; D005944:Glucosamine / Q000378:metabolism; D006023:Glycoproteins / Q000378:metabolism*; D006056:Golgi Apparatus / Q000378:metabolism; D006801:Humans; D007421:Intestine, Small / Q000166:cytology / Q000378:metabolism* / Q000648:ultrastructure; D007583:Jejunum / Q000378:metabolism / Q000648:ultrastructure; D008247:Lysosomes / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D016532:Mucopolysaccharidosis II / Q000378:metabolism / Q000473:pathology
D006023:Glycoproteins; D005643:Fucose; D005944:Glucosamine