Defects in activator proteins and other soluble proteins of the lysosome
This chapter discusses enzymatically inactive proteins that play a part in two areas of endosomal-lysosomal membrane lipid metabolism. Some are involved in binding and transfer processes that assist lysosomal sphingolipid catabolism and others appear to regulate cholesterol transport from these acidic compartments back to the plasma membrane and the endoplasmic reticulum (ER). These activator-mediated processes are of physiological relevance since defects in activator function can lead to fatal human diseases.
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