Molecular Basis of Lysosomal Storage Disorders

Content: 
Front Matter, Page iii
Copyright, Page iv
Contributors, Pages ix-xiv
Preface, Page xv
Introduction, Pages xvii-xix
OLD DIVERSIONS AND NEW DIRECTIONS IN ENZYME PURIFICATION, Pages 3-17, D. Robinson
FUNCTION AND RELEVANCE OF ACTIVATOR PROTEINS FOR GLYCOLIPID DEGRADATION, Pages 19-49, Konrad Sandhoff
CEREBROSIDE SULFATASE ACTIVATOR DEFICIENCY, Pages 51-60, Arvan L. Fluharty, Hayato Kihara
STUDIES ON THE SPHINGOLIPID ACTIVATOR PROTEIN FOR THE ENZYMATIC HYDROLYSIS OF GM1 GANGLIOSIDE AND SULFATIDE, Pages 61-78, David A. Wenger, Koji Inui
THE OCCURRENCE AND PHYSIOLOGICAL SIGNIFICANCE OF ACTIVATOR PROTEINS ESSENTIAL FOR THE ENZYMIC HYDROLYSIS OF GM1 AND GM2 GANGLIOSIDES, Pages 79-91, Yu-Teh Li, Su-Chen Li
THE COHYDROLASES FOR CEREBROSIDE β-GLUCOSIDASE, Pages 93-112, Norman S. Radin
COMBINED β-GALACTOSIDASE-SIALIDASE DEFICIENCY IN MAN: GENETIC DEFECT OF A “PROTECTIVE PROTEIN”, Pages 113-131, Hans Galjaard, Alessandra d'Azzo, André Hoogeveen, Frans Verheijen
LYSOSOMAL STORAGE DISORDERS CAUSED BY INSTABILITY OF THE MISSING ENZYMES, Pages 133-146, Kurt von Figura, Andrej Hasilik, Friedhelm Steckel
Asialoglycoprotein Catabolism by Hepatocytes: Insights from Perturbation of the Endocytic Pathway, Pages 149-162, Joe Harford, Richard D. Klausner, Allan W. Wolkoff, Kenneth R. Bridges, Gilbert Ashwell
THE ROLE OF ACIDIFICATION IN TRANSPORT OF ACID HYDROLASES TO LYSOSOMES, Pages 163-174, William S. Sly M.D., Jeffrey H. Grubb, Joan M. Moehring, Thomas J. Moehring
EVIDENCE FOR A MANNOSE 6-PHOSPHATE-INDEPENDENT PATHWAY FOR LYSOSOMAL ENZYME TARGETING, Pages 175-193, Christopher A. Gabel, Daniel E. Goldberg, Stuart Kornfeld
Adsorptive Pinocytosis and Intracellular Transport of Lysosomal Enzymes: The Role of the Phosphomannosyl Receptor, Pages 195-208, George W. Jourdian, Diane Mitchell, Thomas Maler, Jack J. Distler
The Mannose Recognition Pathway-Implications for Lysosome Physiology, Pages 209-218, Philip D. Stahl, Thomas E. Wileman, Virginia L. Shepherd
INTERACTION OF HUMAN PLACENTAL GLUCOCEREBROSIDASE WITH HEPATIC LECTINS, Pages 219-232, F. Scott Furbish, Katherine L. Oliver, Gregory C. Zirzow, Roscoe O. Brady, John A. Barranger
BIOSYNTHESIS OF LYSOSOMAL ENZYMES, Pages 235-249, Ann H. Erickson, Gregory E. Conner, Günter Blobel
DEFECTIVE SYNTHESIS OR MATURATION OF THE α-CHAIN OF β-HEXOSAMINIDASE IN CLASSIC AND VARIANT FORMS OF TAY-SACHS DISEASE, Pages 251-256, Elizabeth F. Neufeld, Alessandra d'Azzo, Richard L. Proia
THE INTERMEDIATE HEXOSAMINIDASES OF PLACENTA AND THEIR RELATIONSHIP TO PRECURSOR PROCESSING, Pages 257-271, J.A. LOWDEN, D. MAHURAN, B. O'DOWD, R. GRAVEL, F. TSUI
PROCESSING OF HUMAN LYSOSOMAL α-GLUCOSIDASE, Pages 273-284, Joseph M. Tager, Ronald Oude Elferink, Arnold J.J. Reuser, John Hilkens, Jo Hilgers
GENETIC HETEROGENEITY IN LYSOSOMAL STORAGE DISORDERS STUDIED BY SOMATIC CELL HYBRIDIZATION, Pages 287-309, Arnold J.J. Reuser
GENETIC HETEROGENEITY OF GAUCHER'S DISEASE, Pages 311-323, John A. Barranger, Gary J. Murray, Edward I. Ginns
CHROMOSOMAL LOCALIZATION OF THE STRUCTURAL GENES ENCODING THE HUMAN LYSOSOMAL HYDROLASES AND THEIR ACTIVATOR AND STABILIZER PROTEINS, Pages 325-363, Kenneth H. Astrin, Robert J. Desnick
STRATEGIES FOR THE MOLECULAR CLONING OF LOW ABUNDANCE MESSENGER RNAs, Pages 365-385, Arthur L. Horwich, Jan P. Kraus, Leon E. Rosenberg
CLONING OF LYSOSOMAL GENES, Pages 387-403, John S. O'Brien, Jeffrey de Wet, Hisao Fukushima, Edward Wilcox, Nazneen Dewji, Juli McGee, Thomas Warner, Akira Yoshida, Arvan Fluharty, Fred Hill, Donald Helinski
cDNA CLONING OF ACID ALPHA-GLUCOSIDASE, Pages 405-408, Ad Konings
BONE MARROW TRANSPLANTATION IN MUCOPOLYSACCHARIDOSES, Pages 411-428, Kenneth Hugh-Jones, John Hobbs, David Chambers, Susan White, Nigel Byrom, Stanley Williamson, John Barrett, Kristin Henry, Desmond Patrick
BONE MARROW TRANSPLANTATION IN SEVERE GAUCHER'S DISEASE: RAPID CORRECTION OF ENZYME DEFICIENCY ACCOMPANIED BY CONTINUED LONG TERM SURVIVAL OF STORAGE CELLS, Pages 429-440, Edward I. Ginns, Daniel B. Caplan, Joel M. Rappeport, John A. Barranger
BONE MARROW TRANSPLANTATION IN THE NORRBOTTNIAN FORM OF GAUCHER DISEASE, Pages 441-459, Lars Svennerholm, Jan-Eric Månsson, Olle Nilsson, Elisabeth Tibblin, Anders Erikson, Carl-Gustav Groth, Göran Lundgren, Olle Ringdén
ENZYME REPLACEMENT IN THE SPHINGOLIPIDOSES, Pages 461-478, Roscoe O. Brady
STATE OF THE ART, Pages 481-494, Roscoe O. Brady, John A. Barranger
Index, Pages 495-502