Evolution of Lysosomal Proteins
Many of us who have studied lysosomal proteins have an interest in defining the molecular cause of one or other lipid storage disease. The pathology associated with the disordered state can provide considerable insights into the normal process. What we now have, in the form of recombinant DNA techniques, is a tool that can illuminate the structure of the proteins involved in lysosomal lipid catabolism, both in the normal and the disease state. This review considers various aspects of the structure and function of these lipid-metabolising enzymes. The theme running through it is the way in which such proteins are related, with the implication that there may be genetically based links of which we are not yet aware. Or, what one might call, the evolution of lysosomal proteins.
KeywordsPolypeptide Lactose Oligosaccharide Lysozyme Hydrolase
Unable to display preview. Download preview PDF.
- Carroll, M. & Robinson, D., 1974, A low-molecular weight protein crossreacting with human liver N-acetyI-beta-D-gIucosaminidase, Biochem. J., 137:217.Google Scholar
- D’Azzo, A., Hoogeveen, A., Reuser, A. J. J., Robinson, D. & Galjaard, H., 1982, Molecular defect in combined beta-gaIactosidase and neuraminidase deficiency in man, Proc. Nat. Acad. Sci. USA. 79:4534.Google Scholar
- Dean, R.T., 1977, “Lysosomes”,p.12, Edward Arnold, London.Google Scholar
- Geiger, B. & Arnon, R., 1976, Chemical characterisat ion and subunit structure of human N-acetyIhexosaminisases A and B, Biochemistry, 15:3484.Google Scholar
- Kaplan, A., Fischer, D., Achard, D. & Sly, W., 1977, PhosphohexosyI recognition is a general characteristic of pinocytosis of lysosomal glycosidases by human fibroblasts, J.CI in.Invest. 60: 1088.Google Scholar
- Leroy, J.G., Ho, M.W., MacBrinn, M.C., Zielke, K., Jacobs, J. & O’Brien, J. 1972, I-cell disease: biochemical studies, Pediat. Res. 16:752.Google Scholar
- Myerowitz, R., Pickarz, R., Neufeld, E.F., Shows, T.B. & Suzuki, K., 1985, Human beta-hexosaminidase a Ipha-chain:coding sequence and homology with the beta-chain, Proc. Nat. Acad. Sci. USA. 82:7830.Google Scholar
- O’Dowd, B., Quan, F., Willard, H., Lamhonwak, A.M., Korneluk, R., Lowden, Gravel,R.A. & Mahuran, D., 1985, Isolation of cDNA clones coding for the beta-subunit of human beta-hexosaminisase, Proc. Nat. Acad. Sci. USA. 82:1184.Google Scholar
- Robinson, D. & Carroll, M., 1972, Tay-Sachs disease: interreIation of hexosaminisases A and B, Lancet 1: 322.Google Scholar
- Rupley, J.A. & Gates, V., 1967, Studies on the enzymic activity of lysozyme Proc. Nat. Acad. Sci. USA. 57:496.Google Scholar
- Stirling, J. L., 1974, NNf-diacetyIchitobiase activity in Tay-Sachs disease and Sandhoff’s disease. Biochem. J. 141:597.Google Scholar