The strategic function of the P5-ATPase ATP13A2 in toxic waste disposal.
Neurochem Int
; 112: 108-113, 2018 01.
Article
em En
| MEDLINE
| ID: mdl-29169913
ABSTRACT
The P-type ATPase ATP13A2 protein was originally associated with a form of Parkinson's Disease (PD) known as Kufor Rakeb Syndrome (KRS). However, in the last years it has been found to underlay variants of neuronal ceroid-lipofuscinoses and hereditary spastic paraplegia. These findings expand the clinical and genetic spectrum of ATP13A2-associated disorders, which are commonly characterized by lysosomal dysfunction. Nowadays it is well known that lysosomes are not merely related to the degradation and recycling of cellular waste, but are also involved in fundamental processes such as secretion, plasma membrane repair, signaling, energy metabolism and autophagy. The essential role of lysosomes in these cellular processes has significant implications for health and disease. ATP13A2 is localized in lysosomes and late endosomes and its mutation leads to lysosome dysfunction, diminishes the exosome secretion and impairs autophagic flux. In this review, we first describe ATP13A2-associated disorders and their relation with the endolysosomal pathway. We then describe the ATP13A2-involvement in iron homeostasis and its potential linkage with new pathologies like cancer, and finally, we consider the putative role of ATP13A2 in lipid processing and degradation, opening the interesting possibility of a broader role of this protein providing protection against a variety of disease-associated changes affecting cellular homeostasis.
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Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Endossomos
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ATPases Translocadoras de Prótons
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Doenças Neurodegenerativas
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Lisossomos
Limite:
Animals
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Humans
Idioma:
En
Revista:
Neurochem Int
Ano de publicação:
2018
Tipo de documento:
Article