Neuronal Protein Trafficking in Alzheimer's Disease and Niemann-Pick Type C Disease

摘要: Publisher Summary Despite the differences, Alzheimer's disease (AD)—a chronic neurodegenerative of late age—and Niemann–Pick type C (NPC)—an inherited storage disorder early childhood—share pathological features including neurodegeneration, neurofibrillary abnormalities, and P-amyloid accumulation. In both disorders, endocytic pathway function is prominently impaired. Moreover, onset promoted by inheritance ɛ4 isoform apolipoprotein-E, a major carrier protein for cholesterol, suggesting that cholesterol mishandling plays role in AD as well NPC. This chapter reviews cell biology underlying defects these disorders with special emphasis on dysfunction lysosomal system. The addresses how faulty lipid trafficking alterations Rab proteins associated early- late-stage compartments—that is, Rabs 4, 7, 9—may disrupt vesicular transport leading to altered sorting, accumulation endosomal cargos, impaired net movement compartments. redistribution proteases among different types organelles its relevance metabolism amyloid-β precursor formation are also considered chapter.