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.
KeywordsLysosomal Enzyme Beta Subunit Acid Hydrolase Metachromatic Leukodystrophy Lysosomal Protein
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