Incomplete mitophagy in the mevalonate kinase-deficient Saccharomyces cerevisiae and its relation to the MKD-related autoinflammatory disease in humans.
Biochim Biophys Acta Mol Basis Dis
; 1867(4): 166053, 2021 04 01.
Article
em En
| MEDLINE
| ID: mdl-33385519
ABSTRACT
Mevalonate kinase deficiency (MKD) is an autosomal recessive disorder in humans that causes systemic autoinflammatory problems to children. Previously, we used a yeast model to show that MKD results in mitochondrial malfunctioning that may finally induce mitophagy. Here, we proved that MKD indeed induced general autophagy as well as mitophagy in yeast, but these mechanisms did not go to completion. Therefore, the limitation of mevalonate kinase activity produces dysfunctional mitochondria that might not be recycled, causing metabolic dysfunctions in the cells. Understanding this mechanism may provide a piece in solving the nonspecific autoinflammatory response puzzle observed in MKD patients.
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MEDLINE
Assunto principal:
Saccharomyces cerevisiae
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Fosfotransferases (Aceptor do Grupo Álcool)
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Proteínas de Saccharomyces cerevisiae
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Deficiência de Mevalonato Quinase
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Mitofagia
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article