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- W2894715948 endingPage "1238" @default.
- W2894715948 startingPage "1225" @default.
- W2894715948 abstract "Mitochondria are essential organelles which perform complex and varied functions within eukaryotic cells. Maintenance of mitochondrial health and functionality is thus a key cellular priority and relies on the organelle's extensive proteome. The mitochondrial proteome is largely encoded by nuclear genes, and mitochondrial proteins must be sorted to the correct mitochondrial sub-compartment post-translationally. This essential process is carried out by multimeric and dynamic translocation and sorting machineries, which can be found in all four mitochondrial compartments. Interestingly, advances in the diagnosis of genetic disease have revealed that mutations in various components of the human import machinery can cause mitochondrial disease, a heterogenous and often severe collection of disorders associated with energy generation defects and a multisystem presentation often affecting the cardiovascular and nervous systems. Here, we review our current understanding of mitochondrial protein import systems in human cells and the molecular basis of mitochondrial diseases caused by defects in these pathways." @default.
- W2894715948 created "2018-10-12" @default.
- W2894715948 creator A5042624738 @default.
- W2894715948 creator A5051584878 @default.
- W2894715948 creator A5052343612 @default.
- W2894715948 date "2018-10-04" @default.
- W2894715948 modified "2023-09-23" @default.
- W2894715948 title "Mitochondrial diseases caused by dysfunctional mitochondrial protein import" @default.
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