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Mitochondrial protein transport in health and disease
- Source :
- Seminars in Cell & Developmental Biology. 76:142-153
- Publication Year :
- 2018
- Publisher :
- Elsevier BV, 2018.
-
Abstract
- Mitochondria are fundamental structures that fulfil important and diverse functions within cells, including cellular respiration and iron-sulfur cluster biogenesis. Mitochondrial function is reliant on the organelles proteome, which is maintained and adjusted depending on cellular requirements. The majority of mitochondrial proteins are encoded by nuclear genes and must be trafficked to, and imported into the organelle following synthesis in the cytosol. These nuclear-encoded mitochondrial precursors utilise dynamic and multimeric translocation machines to traverse the organelles membranes and be partitioned to the appropriate mitochondrial subcompartment. Yeast model systems have been instrumental in establishing the molecular basis of mitochondrial protein import machines and mechanisms, however unique players and mechanisms are apparent in higher eukaryotes. Here, we review our current knowledge on mitochondrial protein import in human cells and how dysfunction in these pathways can lead to disease.
- Subjects :
- 0301 basic medicine
Cell Biology
Biology
Mitochondrion
Cell biology
Mitochondrial Proteins
Protein Transport
03 medical and health sciences
Mitochondrial membrane transport protein
030104 developmental biology
mitochondrial fusion
Biochemistry
Translocase of the inner membrane
DNAJA3
biology.protein
Humans
Mitochondrial fission
ATP–ADP translocase
Developmental Biology
HSPA9
Subjects
Details
- ISSN :
- 10849521
- Volume :
- 76
- Database :
- OpenAIRE
- Journal :
- Seminars in Cell & Developmental Biology
- Accession number :
- edsair.doi.dedup.....ba0f522ca72d01a385deda8629eb674a