Lysosomal dysfunction in neurodegeneration
نویسندگان
چکیده
منابع مشابه
Lysosomal dysfunction disrupts presynaptic maintenance and restoration of presynaptic function prevents neurodegeneration in lysosomal storage diseases
Lysosomal storage disorders (LSDs) are inherited diseases characterized by lysosomal dysfunction and often showing a neurodegenerative course. There is no cure to treat the central nervous system in LSDs. Moreover, the mechanisms driving neuronal degeneration in these pathological conditions remain largely unknown. By studying mouse models of LSDs, we found that neurodegeneration develops progr...
متن کاملLysosomal Storage Diseases-Regulating Neurodegeneration.
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 by aberrant, excessive storage of cellular material in lysosomes, developed following the discovery of an enzyme deficiency as the cause of Pompe disease in 1963. Great strides have since been m...
متن کاملMitochondrial Dysfunction in Neurodegeneration
The mitochondrial respiratory chain (RC) is responsible for providing most of the cellular energy in the form of ATP, and is also one of the main sites of reactive oxygen species (ROS) formation. The RC, consisting of five enzyme complexes in the inner mitochondrial membrane, is unique in its bipartite dependence on both nuclear and mitochondrial DNA (mtDNA). One example of this is mitochondria...
متن کاملTopic: Mitochondrial Dysfunction in Neurodegeneration
Neurodegenerative diseases such as Parkinson's disease (PD) are of growing public health concern in developed countries and the need for novel effective treatments continues to increase. Neurodegenerative diseases take many forms, reflecting the degeneration of different populations of neurons at different times from distinct causes, but many of them also share common features. Amongst the comm...
متن کاملMitochondrial Dysfunction in Lysosomal Storage Disorders
Lysosomal storage diseases (LSDs) describe a heterogeneous group of rare inherited metabolic disorders that result from the absence or loss of function of lysosomal hydrolases or transporters, resulting in the progressive accumulation of undigested material in lysosomes. The accumulation of substances affects the function of lysosomes and other organelles, resulting in secondary alterations suc...
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ژورنال
عنوان ژورنال: Autophagy
سال: 2012
ISSN: 1554-8627,1554-8635
DOI: 10.4161/auto.20256