The field of lysosomal transport has grown exponentially in the past decade. Research in this previously unknown function of lysosomes has resulted in understanding the metabolic defect in three inborn errors in metabolism: nephropathic cystinosis, cobalamin F-deficient methylmalonic aciduria, and Salla disease. Seventeen transport systems mediating the exodus from lysosomes of amino acids, sugars, nucleosides, phosphate, calcium, cobalamin, and sulfate have been described. Pathophysiology of Lysosomal Transport presents the current status in this field as described by the authors who made the original discoveries. Each chapter examines the pathological consequences resulting from a defect in a particular system. The book also examines the transfer of macromolecules into the lysosomes, describes the analogy between mammalian lysosomes and vacuoles of plants and fungi, and reviews non-mediated transport. A comprehensive chapter on the methodology required to perform lysosomal studies will benefit researchers undertaking investigations in this area.The isomerization of the empty carrier is shown across the center of the diagram. An additional pathway, not physically different from that followed by S, is shown for S* for use in a later discussion of substrate which is isotopically labeled or isanbsp;...
|Title||:||Pathophysiology of Lysosomal Transport|
|Author||:||Jess G. Thoene|
|Publisher||:||CRC Press - 1992-05-12|