The transport of lysosomal enzymes.

This paper reviews the experimental evidence for the proposal that hydrolytic enzymes are introduced into lysosomes of cultured fibroblasts only after secretion and receptormediated recapture.

[1]  M. Rattazzi,et al.  Abnormal lysosomal hydrolases excreted by cultured fibroblasts in I-cell disease (mucolipidosis II). , 1975, Biochemical and biophysical research communications.

[2]  P. Schlesinger,et al.  Evidence for specific recognition sites mediating clearance of lysosomal enzymes in vivo. , 1976, Proceedings of the National Academy of Sciences of the United States of America.

[3]  A. Morell,et al.  The existence of a second route for the transfer of certain glycoproteins from the circulation into the liver. , 1976, Biochemical and biophysical research communications.

[4]  P. Schlesinger,et al.  Clearance of lysosomal hydrolases following intravenous infusion: The role of liver in the clearance of β-glucuronidase and N-acetyl-β-d-glucosaminidase , 1976 .

[5]  A. Miller,et al.  Sanfilippo Disease Type B: Enzyme Replacement and Metabolic Correction in Cultured Fibroblasts , 1973, Science.

[6]  S. Didonato,et al.  Effect of chloroquine on cultured fibroblasts: release of lysosomal hydrolases and inhibition of their uptake. , 1975, Biochemical and biophysical research communications.

[7]  J. Goust,et al.  Immunologic abnormalities in subacute sclerosing panencephalitis. , 1971, The New England journal of medicine.

[8]  G. Thomas,et al.  Analysis of N-acetyl- -D-glucosaminidase in mucolipidosis II (I-cell disease). , 1973, Clinica chimica acta; international journal of clinical chemistry.

[9]  P. Schlesinger,et al.  Clearance of lysosomal hydrolases following intravenous infusion: Kinetic and competition experiments with β-glucuronidase and N-acetyl-β-d-glucosaminidase☆ , 1976 .

[10]  B. Weissmann,et al.  The defect in the Hurler and Scheie syndromes: deficiency of -L-iduronidase. , 1972, Proceedings of the National Academy of Sciences of the United States of America.

[11]  H. Galjaard,et al.  Intercellular exchange of lysosomal enzymes: enzyme assays in single human fibroblasts after co-cultivation. , 1976, Biochemical and biophysical research communications.

[12]  K. von Figura,et al.  Quantitative aspects of pinocytosis and the intracellular fate of N-acetyl-alpha-D-glucosaminidase in Sanfilippo B fibroblasts. , 1974, The Journal of clinical investigation.

[13]  E. Neufeld,et al.  A recognition marker required for uptake of a lysosomal enzyme by cultured fibroblasts. , 1974, Biochemical and biophysical research communications.

[14]  G. Ashwell,et al.  A hepatic receptor of avian origin capable of binding specifically modified glycoproteins. , 1976, Proceedings of the National Academy of Sciences of the United States of America.

[15]  W. Sly,et al.  Multiple isoelectric and recognition forms of human β-glucuronidase activity , 1975 .

[16]  N. Herschkowitz,et al.  Australia antigen in volunteer and paid blood donors. , 1971 .

[17]  W. Sly,et al.  In vitro correction of deficient human fibroblasts by beta-glucuronidase from different human sources. , 1974, Biochemical and biophysical research communications.

[18]  E. Neufeld,et al.  Inherited disorders of lysosomal metabolism. , 1975, Annual review of biochemistry.

[19]  R. Schmickel,et al.  The role of glycosidically bound mannose in the assimilation of beta-galactosidase by generalized gangliosidosis fibroblasts. , 1976, Biochemical and biophysical research communications.

[20]  C. W. Hall,et al.  The relationship of α-l-iduronidase and Hurler corrective factor , 1976 .

[21]  P. Schlesinger,et al.  Recognition of lysosomal glycosidases in vivo inhibited by modified glycoproteins , 1976, Nature.

[22]  E. Neufeld,et al.  A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes. , 1972, Biochemical and biophysical research communications.

[23]  N. Feder.  Solitary cells and enzyme exchange in tetraparental mice , 1976, Nature.

[24]  C. Duve,et al.  Functions of lysosomes. , 1966, Annual review of physiology.

[25]  G. Thomas,et al.  Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease (mucolipidosis II) fibroblasts. , 1976, Biochemical and biophysical research communications.

[26]  G. Weissmann,et al.  Lysosomes and joint disease. , 1966, Arthritis and rheumatism.

[27]  P. Tulkens,et al.  Immunological Inhibition of Lysosome Function , 1970, Nature.

[28]  C. W. Hall,et al.  α-l-Iduronidase activity in cultured skin fibroblasts and amniotic fluid cells , 1973 .

[29]  B. NovikoffA.  小胞体 細胞化学者の概観(総説) , 1976 .

[30]  A. Novikoff.  The endoplasmic reticulum: a cytochemist's view (a review). , 1976, Proceedings of the National Academy of Sciences of the United States of America.

[31]  D. Nicol,et al.  Differential uptake of human beta-glucuronidase isoenzymes from spleen by deficient fibroblasts. , 1974, Biochemical and biophysical research communications.