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Biosynthesis and Transport of Lysosomal Enzymes

  • Odd Nygård
  • Peter Westermann
Chapter
  • 63 Downloads

Abstract

Lysosomes are digestive organelles found in most eucaryote cells. Typically they are endowed with a large set of mostly hydrolytic enzymes able to break down in concert nearly all biological polymers or complex molecules, which happen to be delivered into these organelles. The digestion depends on acidic conditions within the lysosomes - the internal pH is lower than that in the extralysosomal space by about 2 units (1,2) - and on the delivery into the lysosomes of both the substrates and the enzymes. The substrates are delivered into the lysosomes from the cell interior as well as from exterior. Traditionally,lysosomes were considered to take care of material interiorized by the cells. For recent reviews on endocytosis pathways in eucaryotes the reader may consult ref. 3–5. Today, the participation of lysosomes in the degradation of endogenous proteins is firmly established, too (ref. 6 and references therein). Lysosomal enzymes also participate in the breakdown of extracellular matrix. Some cells like human fibroblasts constitutively secrete a significant fraction of newly synthesized lysosomal enzymes (7).

Keywords

Golgi Apparatus Lysosomal Enzyme Coated Vesicle Mannose Residue Phosphate Residue 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. 1.
    Ohkuma, S. and Poole, B. (1978) Proc. Natl. Acad. Sci. U.S.A. 75, 3327–3331PubMedCrossRefGoogle Scholar
  2. 2.
    Kakinuma, Y., Ohsumi, Y. and Anraku, Y. (1981) J. Biol. Chem. 256, 10859–10863PubMedGoogle Scholar
  3. 3.
    Goldstein, J. L., Anderson, R. G. W. and Brown, M. S. (1979) Nature 279, 679–685PubMedCrossRefGoogle Scholar
  4. 4.
    Besterman, I. M. and Low, R. B. (1983) Biochem. J. 210, 1–13PubMedGoogle Scholar
  5. 5.
    Steinman, R. M., Mellman, I. S., Muller, W. A. and Cohn, Z. A. (1983) J. Cell Biol. 96, 1–27PubMedCrossRefGoogle Scholar
  6. 6.
    Cockle, S. M. and Dean, R. T. (1982) Biochem. J. 208, 795–800PubMedGoogle Scholar
  7. 7.
    Hasilik, A. and Neufeld, E. F. (1980) J. Biol. Chem.255, 4937–4945PubMedGoogle Scholar
  8. 8.
    Page, R. C., Davis, P. and Allison, A. C. (1978) Int. Rev. Cytol. 52, 119–157PubMedCrossRefGoogle Scholar
  9. 9.
    Tulsiani, D. R. P., Keller, R. K. and Touster, D. (1975) J. Biol. Chem. 250, 4770–4776PubMedGoogle Scholar
  10. 10.
    Erickson, A. H. and Blobel, G. (1979) J. Biol. Chem. 254, 11771–11774PubMedGoogle Scholar
  11. 11.
    Erickson, A. H., Conner, G. E. and Blobel, G. (1981) J. Biol. Chem. 256, 11224–11231PubMedGoogle Scholar
  12. 12.
    Rosenfeld, M. G., Kreibich, D., Popov, D., Kato, K. and Sabatini, D. D. (1982) J. Cell Biol. 93, 135–143PubMedCrossRefGoogle Scholar
  13. 13.
    Proia, R. and Neufeld, E. F. (1982) Proc. Natl. Acad. Sci. U.S.A. 79, 6360–6364PubMedCrossRefGoogle Scholar
  14. 14.
    Nichols, B. A., Bainton, D. F. and Farquhar, M. G. (1971) J. Cell Biol. 50, 498–515PubMedCrossRefGoogle Scholar
  15. 15.
    Malley, F. and Trimble, B. B. (1981) J. Biol. Chem. 256, 1088–1090Google Scholar
  16. 16.
    Tkacz, J. and Lampen, J. O. (1975) Biochem. Biophys. Res. Commun. 65, 248–257PubMedCrossRefGoogle Scholar
  17. 17.
    Lehle, L. and Tanner, W. (1976) FEBS Letters 71, 167–170CrossRefGoogle Scholar
  18. 18.
    Olden, K., Parent, B. and White, S. L. (1982) Biochem. Biophys. Acta 650, 209–232PubMedGoogle Scholar
  19. 19.
    Gibson, R., Kornfeld, S. and Schlesinger, S. (1980) Trends Biochem. Sci. 5, 290–293CrossRefGoogle Scholar
  20. 20.
    von Figura, K., Hasilik, A., Waheed, A. and Klein, U. (1981) Perspect. Inherit. Metabol. Dis. 4, 463–468Google Scholar
  21. 21.
    Hubbard, S. C. and Iwatt, R. J. (1981) Annu. Rev. Biochem. 50, 555–583PubMedCrossRefGoogle Scholar
  22. 22.
    Struck, D. K. and Lennarz, W. J. (1980) in: The Biochemistry of Glycoproteins and Proteoglycans (W. J.Lennarz, ed.) pp 35–83, Plenum Press, New YorkGoogle Scholar
  23. 23.
    Sharon, N. and Lis, H. (1980) in: The Proteins, Vol. 5, 3rd Edn. ( H. Neurath and R. C. Hill, eds.) Acad. Press, New YorkGoogle Scholar
  24. 24.
    Kiely, M. C., McKnight, G. S. and Schimke, R. T. (1976) J. Biol. Chem. 251, 5490–5495PubMedGoogle Scholar
  25. 25.
    Lingappa, V. R., Katz, F. N., Lodish, H. F. and Blobel, G. (1978) J. Biol. Chem. 253, 8667–8670PubMedGoogle Scholar
  26. 26.
    Bielinska, M. and Boime, I. (1979) Proc. Natl. Acad. Sci. U.S.A. 76, 1208–1212PubMedCrossRefGoogle Scholar
  27. 26.
    Bielinska, M. and Boime, I. (1979) Proc. Natl. Acad. Sci. U.S.A. 76, 1208–1212PubMedCrossRefGoogle Scholar
  28. 28.
    Rothman, J. E. (1981) Science 213, 1212–1219PubMedCrossRefGoogle Scholar
  29. 29.
    Mizuochi, T., Nishimura, Y., Kato, K. and Kobata, A. (1981) Arch. Biochem. Biophys. 209, 298–303PubMedCrossRefGoogle Scholar
  30. 30.
    Hasilik, A., Klein, U., Waheed, A., Strecker, G. and von Figura, K. (1980) Proc. Natl. Acad. Sci. U.S.A. 77, 7074–7078PubMedCrossRefGoogle Scholar
  31. 31.
    Tabas, I. and Kornfeld, S. (1980) J. Biol. Chem. 255, 6633–6639PubMedGoogle Scholar
  32. 32.
    Goldberg, D. E. and Kornfeld, S. (1981) J. Biol. Chem. 256, 13060–13067PubMedGoogle Scholar
  33. 33.
    Varki, A. and Kornfeld, S. (1980) J. Biol. Chem. 255, 10847–10858PubMedGoogle Scholar
  34. 34.
    Hasilik, A. and von Figura, K. (1981) Eur. J. Biochem. 121, 125–129PubMedCrossRefGoogle Scholar
  35. 35.
    Roth, J. and Berger, E. G. (1982) J. Cell Biol. 93, 223–229PubMedCrossRefGoogle Scholar
  36. 36.
    Dunphy, W. G., Fries, E., Urbani, L. J. and Rothman,J.E. (1981) Proc. Natl. Acad. Sci. U.S.A. 78, 7453–7457PubMedCrossRefGoogle Scholar
  37. 37.
    Quinn, P., Griffith, G. and Warren, G. (1983) J. Cell Biol. 96, 851–856PubMedCrossRefGoogle Scholar
  38. 38.
    Sly, W. S., Gonzales-Noricga, A., Natowicz, M.F Fisher, H. D. and Chambers, J. P. (1979) Feder. Proc. 38, 1256Google Scholar
  39. 39.
    Miller, A. L., Kress, B. C., Stein, R., Kinnon, C., Kern, H., Schneider, A. and Harms, E. (1981) J. Biol. Chem.256, 9352–9362PubMedGoogle Scholar
  40. 40.
    Neufeld, E. F. and Ashwell, G. (1980) in: The Biochemistry of Glycoproteins and Proteoglycans (W.J.Lennarz, ed.) pp 241–266, Plenum Press, New YorkGoogle Scholar
  41. 41.
    Hickman, S., Shapiro, L. J. and Neufeld, E. F. (1974) Biochem. Biophys. Res. Commun. 57, 55–61PubMedCrossRefGoogle Scholar
  42. 42.
    Hieber, V., Distler, J., Myerowitz, R., Schinickel, R.D. and Jourdian, G. W. (1976) Biochem. Biophys. Res.Coinmun.73, 710–717CrossRefGoogle Scholar
  43. 43.
    Kaplan, A., Achord, D. T. and Sly, W. S. (1977) Proc. Natl. Acad. Sci. U.S.A.74, 2026–2030PubMedCrossRefGoogle Scholar
  44. 44.
    Sando, G. N. and Neufeld, E. F. (1977) Cell 12, 619–627PubMedCrossRefGoogle Scholar
  45. 45.
    Kaplan, A., Fischer,D., Achord, D. T. and Sly, W. S. (1977) J. Clin. Invest.60, 1088–1093PubMedCrossRefGoogle Scholar
  46. 46.
    Ullrich, K., Mersmann, G., Weber, R. and von Figura, K. (1977) Biochem. J.170, 643–650Google Scholar
  47. 47.
    Kaplan, A., Fischer, D. and Sly, W. S. (1978) J. Biol. Chem.253, 647–650PubMedGoogle Scholar
  48. 48.
    Hasilik, A. and Neufeld, E. F. (1980) J. Biol. Chem. 255, 4946–4950PubMedGoogle Scholar
  49. 49.
    Natowicz, M. R., Chi, M. M. Y., Lowry, O. H. and Sly, W. S. (1979) Proc. Natl. Acad. Sci. U.S.A. 76, 4322–4326PubMedCrossRefGoogle Scholar
  50. 50.
    Bach, G., Bargal, R. and Cantz, M. (1979) Biochem. Biophys. Res. Commun. 91, 976–981PubMedCrossRefGoogle Scholar
  51. 51.
    Karson, E. M. and Ballou, C. E. (1978) J. Biol. Chem. 253, 6484–6492PubMedGoogle Scholar
  52. 52.
    Hasilik, A., Waheed, A. and von Figura, K. (1981) Biochem. Biophys. Res. Commun. 98, 761–767PubMedCrossRefGoogle Scholar
  53. 53.
    Reitman, M. L. and Kornfeld, S. (1981) J. Biol. Chem. 256, 4275–4281PubMedGoogle Scholar
  54. 54.
    Waheed, A., Hasilik, A., Cantz, M. and von Figura, K. (1981) Hoppe-Seyler’s Z. Physiol. Chem. 363, 169–178CrossRefGoogle Scholar
  55. 55.
    Waheed, A., Hasilik, A. and von Figura, K. (1982) J. Biol. Chem. 257, 12322–12331PubMedGoogle Scholar
  56. 56.
    Reitman, M. L. and Kornfeld, S. (1981) J. Biol. Chem. 256, 11977–11980PubMedGoogle Scholar
  57. 57.
    Owada, M. and Neufeld, E. F. (1982) Biochem. Biophys. Res. Commun. 105, 814–820PubMedCrossRefGoogle Scholar
  58. 58.
    Natowicz, M., Baenziger, J. U. and Sly, W. S. (1982) J. Biol. Chem. 257, 4412–4420PubMedGoogle Scholar
  59. 59.
    Gabel, C. A., Goldberg, D. F. and Kornfeld, S. (1982) J. Cell Biol. 95, 536–542PubMedCrossRefGoogle Scholar
  60. 60.
    Varki, A. and Kornfeld, S. (1980) J. Biol. Chem. 255, 8398–8401PubMedGoogle Scholar
  61. 61.
    Waheed, A., Hasilik, A. and von Figura, K. (1981) J.Biol. Chem. 256, 5717–5721PubMedGoogle Scholar
  62. 62.
    Varki, A. and Kornfeld, S. (1981) J. Biol. Chem. 256, 9937–9943PubMedGoogle Scholar
  63. 63.
    Waheed, A., Pohlnvann, R., Hasilik, A. and von Figura, K. (1981) J. Biol. Chem. 256, 4150–4152PubMedGoogle Scholar
  64. 64.
    Pohlmanh, R., Waheed, A., Hasilik, A. and von Figura, K. (1982) J. Biol. Chem. 257, 5323–5325Google Scholar
  65. 65.
    Tabas, I. and Kornfeld, S. (1979) J. Biol. Chem. 254, 11655–11663PubMedGoogle Scholar
  66. 66.
    Gabel, C. A. and Kornfeld, S. (1982) J. Biol. Chem. 257, 10605–10612PubMedGoogle Scholar
  67. 67.
    Varki, A. and Kornfeld, S. (1983) J. Biol. Chem. 258, 2808–2818PubMedGoogle Scholar
  68. 68.
    Rome, L. H., Weissmann, B. and Neufeld, E. F. (1979) Proc. Natl. Acad. Sci. U.S.A. 76, 2331–2334PubMedCrossRefGoogle Scholar
  69. 69.
    Gonzales-Noriega, A., Grubb, J. H., Talkad, V. and Sly, W. S. (1980) J. Cell Biol. 85, 839–852; Fischer, H. D., Gonzales-Noriega, A. and Sly, W. S. (1980) J. Biol.Chem. 255, 5069–5074Google Scholar
  70. 70.
    Sly, W. S. and Fischer, H. D. (1982) J. Cell Biochem. 78, 531–549Google Scholar
  71. 71.
    Sahagian, G. G., Distler, J. and Jourdian, G. W. (1981) Proc. Natl. Acad. Sci. U.S.A. 78, 4289–4293PubMedCrossRefGoogle Scholar
  72. 72.
    Steiner, A. W. and Rome, L. H. (1982) Arch. Biochem. Biophys. 214, 681–687PubMedCrossRefGoogle Scholar
  73. 73.
    Sahagian, G. G., Distler, J. and Jourdian, G. W. (1982) Methods Enzymol. 83, 392–396PubMedCrossRefGoogle Scholar
  74. 74.
    Fischer, H. D., Gonzales-Noriega, A., Sly, W. S. and Morre, D. J. (1980) J. Biol. Chem. 255, 9608–9615PubMedGoogle Scholar
  75. 75.
    Campbell, C. H., Fine, R. E., Squicciarini, J. and Rome, L. H. (1983) J. Biol. Chem. 258, 2628–2633PubMedGoogle Scholar
  76. 76.
    Brown, M. S., Anderson, R. G. W. and Goldstein, J. L. (1983) Cell 32, 663–667PubMedCrossRefGoogle Scholar
  77. 77.
    Creek, K. E. and Sly, W. S. (1982) J. Biol. Chem. 257, 9931–9937PubMedGoogle Scholar
  78. 78.
    Natowicz, M., Hallett, D. N., Frier, C., Chi, M., Schlesinger, P. H. and Baenziger, J. U. (1983) J. Cell Biol. 96, 915–919PubMedCrossRefGoogle Scholar
  79. 79.
    Fischer, H. D., Creek, K. E. and Sly, W. S. (1982) J. Biol. Chem. 257, 9939–9943Google Scholar
  80. 80.
    Willingham, M. C., Pastan, I. H., Sahagian, G. G., Jourdian, G. W. and Neufeld, E. F. (1981) Proc. Natl. Acad. Sci. U.S.A. 78, 6967–6971PubMedCrossRefGoogle Scholar
  81. 81.
    Rome, L. H., Garvin, A. J., Alietta, M. M. and Neufeld, E. F. (1979) Cell 16, 143–153CrossRefGoogle Scholar
  82. 82.
    Goldberg, D. E. and Kornfeld, S. (1983) J. Biol. Chem. 258, 3159–3165PubMedGoogle Scholar
  83. 83.
    Geuze, H. J., Slot, J. W., Strous, G.J.A.M., Hasilik, A. and von Figura, K. (1983) submittedGoogle Scholar
  84. 84.
    Friend, D. S. and Farquhar, M. G. (1967) J. Cell Biol. 35, 357–376PubMedCrossRefGoogle Scholar
  85. 85.
    Tümmers, S., Zühlsdorf, M., Hasilik, A. and von Figura, K. (1983) submittedGoogle Scholar
  86. 86.
    Wehland, J., Willingham, M. C., Gallo, M. G. and Pastan, I. (1982) 28, 831–841PubMedCrossRefGoogle Scholar
  87. 87.
    Rothman, J. E. and Fine, R. E. (1980) Proc. Natl. Acad. Sci. U.S.A. 77, 780–784PubMedCrossRefGoogle Scholar
  88. 88.
    Tycko, B. and Maxfield, F. R. (1982) Cell 28, 643–651PubMedCrossRefGoogle Scholar
  89. 89.
    Forgac, M., Cantley, L., Wiedenmann, B., Altstiel, L. and Branton, D. (1983) Proc. Natl. Acad. Sci. U.S.A. 80, 1300–1303PubMedCrossRefGoogle Scholar
  90. 90.
    Marsh, M., Bolzan, E. and Helenius, A. (1983) Cell 32, 931–940PubMedCrossRefGoogle Scholar
  91. 91.
    Bridges, K., Harford, J., Ashwell, G. and Klausner,R.D. (1982) Proc. Natl. Acad. Sci. U.S.A. 79, 350–354PubMedCrossRefGoogle Scholar
  92. 92.
    Robbins, A. R., Myerowitz, R., Youle, R. J., Murray,G.J. and Neville, D. M. Jr. (1981) J. Biol. Chem. 256, 10618–10622PubMedGoogle Scholar
  93. 93.
    Robbins, A. R. and Myerowitz, R. (1981) J. Biol. Chem. 256, 10623–10627PubMedGoogle Scholar
  94. 94.
    Gabel, C. A., Goldberg, D. E. and Kornfeld, S. (1983) Proc. Natl. Acad. Sci. U.S.A. 80, 775–779PubMedCrossRefGoogle Scholar
  95. 95.
    Reitman, M. L., Varki, A. and Kornfeld, S. (1981) J. Clin. Invest. 67, 1574–1579PubMedCrossRefGoogle Scholar
  96. 96.
    Hasilik, A., Waheed, A., Cantz, M. and von Figura, K. (1982) Eur. J. Biochem. 122, 119–123PubMedCrossRefGoogle Scholar
  97. 97.
    Robey, P. G. and Neufeld, E. F. (1982) Arch. Biochem. Biophys. 213, 251–257PubMedCrossRefGoogle Scholar
  98. 98.
    Gilbert, E. F., Dawson, G., ZuRhein, G. M., Opitz,J.M. and Spranger, J. W. (1973) Z. Kinderheilk.114, 259–292PubMedCrossRefGoogle Scholar
  99. 99.
    Waheed, A., Pohlmann, R., Hasilik, A., von Figura, K., Elsen, A. and Leroy, J. G. (1981) Biochem. Biophys.Res. Commun. 105, 1052–1058CrossRefGoogle Scholar
  100. 100.
    Hasilik, A., Pohlmann, R. and von Figura, K. (1983) Biochem. J. 210, 795–802PubMedGoogle Scholar
  101. 101.
    Pohlmann, R., Krüger, S., Hasilik, A. and von Figura,K. unpublishedGoogle Scholar
  102. 102.
    Imort, M., Zühlsdorf, M., Feige, U., Hasilik, A. and von Figura, K. (1983) Biochem. J., in pressGoogle Scholar
  103. 103.
    Tolstoshev, P., Berg, R. A., Rennard, R. I., Bradley, K. H., Trapnell, B. C. and Crystal, R. G. (1981) J. Biol. Chem. 256, 3135–3140PubMedGoogle Scholar
  104. 104.
    Berg, R. A., Schwartz, M. L. and Crystal, R. G. (1980) Proc. Natl. Acad. Sci. U.S.A. 77, 4746–4750PubMedCrossRefGoogle Scholar
  105. 105.
    Diegelraann, R. F., Cohen, I. K. and Guzelian, P. S. (1980) Biochem. Biophys. Res. Commun. 97, 819–826CrossRefGoogle Scholar
  106. 106.
    Hashimoto, C., Cohen, R. E., Zhany, W.-J. and Ballou, C. E. (1981) Proc. Natl. Acad. Sci. U.S.A. 78, 2244–2248PubMedCrossRefGoogle Scholar
  107. 107.
    Schwaiger, H., Hasilik, A., von Figura, K., Wiemken,A. and Tanner, K. (1982) Biochem. Biophys. Res. Commun. 104, 950–956PubMedCrossRefGoogle Scholar
  108. 108.
    Onishi, H. R., Tkacz, J. S. and Lampen, J. O. (1979) J. Biol. Chem. 254, 11943–11952PubMedGoogle Scholar
  109. 109.
    Stevens, T., Esmon, B. and Scheckman, R. (1982) Cell 30, 439–448PubMedCrossRefGoogle Scholar
  110. 110.
    Hasilik, A. and Tanner, W. (1976) Biochem. Biophys. Res. Commun. 72, 1430–1436PubMedCrossRefGoogle Scholar
  111. 111.
    Hasilik, A. (1980) Trends Biochem. Sci. 5, 237–240CrossRefGoogle Scholar
  112. 112.
    Neufeld, E. F. (1981) in: Lysosomes and Lysosomal Storage Diseases (J. W. Callahen and J. A. Lowden, eds.) pp 131–146, Raven Press, New YorkGoogle Scholar
  113. 113.
    Hasilik, A. and von Figura, K. (1983) in: Lysosomes in Biology and Pathology (R. T. Dean and J. T. Dingle,) Vol. 7, Elsevier/North Holland, Amsterdam, in pressGoogle Scholar
  114. 114.
    Skudlarek, M. D. and Swank, R. T. (1979) J. Biol. Chem. 254, 9939–9942PubMedGoogle Scholar
  115. 115.
    Skudlarek, M. D. and Swank, R. T. (1981) J. Biol.Chem. 256, 10137–10144PubMedGoogle Scholar
  116. 116.
    Frisch, A. and Neufeld, E. F. (1981) J. Biol. Chem. 256, 8242–8246PubMedGoogle Scholar
  117. 117.
    Steckel, F., Hasilik, A. and von Figura, K. (1983) submittedGoogle Scholar
  118. 118.
    Hasilik, A., von Figura, K., Conzelmann, E., Nehrkorn, H. and Sandhoff, K. (1982) Eur. J. Biochem. 125, 317–321PubMedCrossRefGoogle Scholar
  119. 119.
    Hemmings, B. A., Zubenko, G. S., Hasilik, A. and Jones, E. W. (1981) Proc. Natl. Acad. Sci. U.S.A. 78, 435–439PubMedCrossRefGoogle Scholar
  120. 120.
    Zubenko, G. S., Park, F. J. and Jones, E. W. (1983) Proc. Natl. Acad. Sci. U.S.A. 80, 510–514PubMedCrossRefGoogle Scholar
  121. 121.
    Moore, H.-P., Gumbiner, B. and Kelly, R. B. (1983) Nature 302, 434–436PubMedCrossRefGoogle Scholar

Copyright information

© The Human Press Inc. 1983

Authors and Affiliations

  • Odd Nygård
    • 1
  • Peter Westermann
    • 2
  1. 1.The Wenner-Gren InstituteUniversity of StockholmStockholmSweden
  2. 2.Central Institute of Molecular BiologyAcademy of Sciences of GDRBerlin-BuchGermany

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