The Pharmacology of Respiratory Care

conference held during October 1979 in Toronto, brings together a wealth of information added to our understanding of the molecular biology of the lysosome. The first two chapters concisely review aspects of the genetics and metabolic regulation of lysosomal enzyme activity. The following group of papers, in logical sequence, trace newer developments in the mechanisms of membrane glycoprotein synthesis and processing, early events in enzyme secretion, and the specialized enzymology of the hexoseaminidases, sulfatases, sphingomyelinases, and glycosidases. The third group of papers, comprising the entire second half of the book, explore a diverse selection of topics on the clinical pathophysiology of inherited lysosome storage defects. Representative syndromes include those of Niemann-Pick, Tay-Sachs, Maroteaux-Lamy, Batten, and Fabrey disease. The final chapters deal with improved antenatal diagnosis, carrier screening programs, and enzyme plasmapheresis and replacement strategies. Overall, this volume is a fascinating and welledited collection of current research trends of certain interest to biochemical and clinical geneticists studying the lysosome. JEFFREY L. NOEBELS Medical Student Yale University School of Medicine