Lysosomes (Google eBook)
Lysosomes are membrane-surrounded organelles which are present in all animal cells. The importance of this organelle is underlined by an increasing number of human diseases, which are associated with an impaired function of the lysosomal compartment. This book summarizes the current state-of-the art knowledge about this unique organelle. It addresses the biogenesis of this compartment, the transport of lysosomal proteins, the role of the lysosomal membrane in lysosomal stability and transport, the function of lysosomal proteases and hydrolases, lysosomal storage disorders, and new concepts on how to treat these diseases. In addition to these classical topics, new insights into lysosomal functions are covered by chapters dealing with specialized lysosomes involved in bone resorption and plasma membrane repair, the lysosomal transciptome, and proteome and the emerging role of lysosomes in special forms of autophagy. This book will provide readers with a comprehensive overview into how this fascinating organelle works and how research in the field is developing.
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History and Morphology of the Lysosome
Transport of Lysosomal Enzymes
Adaptor Proteins in Lysosomal Biogenesis
Lysosomal Membrane Proteins
Revival of the Sleeping Beauty
Lysosomal Storage Disorders
Lysosomal Transporters and Associated Diseases
to Degrade Mineralised Tissues
The Selective Lysosomal Pathway for the Degradation
Acad Sci USA accumulation activity amino acids autophagic vacuoles autophagosomes autophagy Batten disease binding Biochem Biol Chem bone marrow brain cathepsin cathepsin D Cell Biol Cell Sci cellular cholesterol clathrin clinical CLN3 compartment complex cysteine proteinases cystinosis cytoplasmic cytosolic defect deﬁciency degradation domain encoding endocytic endocytosis Enzyme replacement exocytosis expression ﬁbroblasts ﬁrst function gene therapy Genet glycoprotein Golgi granules human hydrolases identiﬁed infantile intracellular late endosomes lipid liver lysosomal enzymes lysosomal membrane proteins lysosomal proteases lysosomal storage diseases lysosomal storage disorders mannose 6-phosphate receptor mediated Metabolic mice molecular mouse model MPRs mucopolysaccharidosis mutations Natl Acad Sci neuronal ceroid lipofuscinosis Niemann-Pick NPC1 NPC2 organelles osteoclasts overexpression pathway patients peptides phenotype plasma membrane Proc Natl Acad proteolytic proteome regulated residues resorption role ruffled border Saftig secretory sequence speciﬁc structure substrate subunit synaptotagmin targeting tissues trafficking transmembrane transplantation vacuoles vector vesicles yeast