Mitochondrial proteases in human diseases
| Autor: | Friederike-Nora Vögtle, Maria Gomez-Fabra Gala |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Proteases
Proteome Proteolysis Biophysics Mitochondrion Biology Biochemistry Mitochondrial Proteins 03 medical and health sciences Structural Biology Genetics medicine Humans Mitochondrial protein processing Molecular Biology 030304 developmental biology 0303 health sciences medicine.diagnostic_test 030302 biochemistry & molecular biology Neurodegeneration Proteolytic enzymes Cell Biology medicine.disease Cell biology Mitochondria Proteostasis Biogenesis Peptide Hydrolases |
| Zdroj: | FEBS lettersReferences. 595(8) |
| ISSN: | 1873-3468 |
| Popis: | Mitochondria contain more than 1000 different proteins, including several proteolytic enzymes. These mitochondrial proteases form a complex system that performs limited and terminal proteolysis to build the mitochondrial proteome, maintain, and control its functions or degrade mitochondrial proteins and peptides. During protein biogenesis, presequence proteases cleave and degrade mitochondrial targeting signals to obtain mature functional proteins. Processing by proteases also exerts a regulatory role in modulation of mitochondrial functions and quality control enzymes degrade misfolded, aged, or superfluous proteins. Depending on their different functions and substrates, defects in mitochondrial proteases can affect the majority of the mitochondrial proteome or only a single protein. Consequently, mutations in mitochondrial proteases have been linked to several human diseases. This review gives an overview of the components and functions of the mitochondrial proteolytic machinery and highlights the pathological consequences of dysfunctional mitochondrial protein processing and turnover. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text