Lysosomal Storage Diseases-Regulating Neurodegeneration.
作者： Rob U. Onyenwoke , Jay E. Brenman
DOI: 10.4137/JEN.S25475
关键词:
摘要: Autophagy is a complex pathway regulated by numerous signaling events that recycles macromolecules and can be perturbed in lysosomal storage diseases (LSDs). The concept of LSDs, which are characterized aberrant, excessive cellular material lysosomes, developed following the discovery an enzyme deficiency as cause Pompe disease 1963. Great strides have since been made better understanding biology LSDs. Defective typically occurs many cell types, but nervous system, including central system peripheral particularly vulnerable to being affected two-thirds This review provides summary some LSDs pathways these disorders.
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