Mitochondrial proteases in human diseases
Abstract: 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
- Standort
-
Deutsche Nationalbibliothek Frankfurt am Main
- Umfang
-
Online-Ressource
- Ausgabe
-
Online first
- Sprache
-
Englisch
- Anmerkungen
-
FEBS letters. - 595, 8 (2021) , 1205-1222, ISSN: 1873-3468
- Ereignis
-
Veröffentlichung
- (wo)
-
Freiburg
- (wer)
-
Universität
- (wann)
-
2021
- Urheber
- DOI
-
10.1002/1873-3468.14039
- URN
-
urn:nbn:de:bsz:25-freidok-1936402
- Rechteinformation
-
Der Zugriff auf das Objekt ist unbeschränkt möglich.
- Letzte Aktualisierung
-
14.08.2025, 10:54 MESZ
Datenpartner
Deutsche Nationalbibliothek. Bei Fragen zum Objekt wenden Sie sich bitte an den Datenpartner.
Beteiligte
- Gomez‐Fabra Gala, Maria
- Vögtle, Friederike-Nora
- Universität
Entstanden
- 2021
Ähnliche Objekte (12)
Intramembrane proteases in neurodegenerative diseases
Autophagy in major human diseases
Mitochondrial protein import clogging as a mechanism of disease
Altered metabolic flexibility in inherited metabolic diseases of mitochondrial fatty acid metabolism
In the moonlight: non-catalytic functions of ubiquitin and ubiquitin-like proteases
Quantitative modeling of human diseases - methods and applications
Open questions on the mitochondrial unfolded protein response
Immunotherapy in myeloproliferative diseases
The evolutionary fate of mitochondrial aminoacyl-tRNA synthetases in amitochondrial organisms
Low level lysosomal membrane permeabilization for limited release and sub‐lethal functions of cathepsin proteases in the cytosol and nucleus
Mitochondrial machineries for protein import and assembly
Fatigue bei chronisch körperlichen Erkrankungen : = Fatigue in chronic physical diseases
Intramembrane proteases in neurodegenerative diseases
Autophagy in major human diseases
Mitochondrial protein import clogging as a mechanism of disease
Altered metabolic flexibility in inherited metabolic diseases of mitochondrial fatty acid metabolism
In the moonlight: non-catalytic functions of ubiquitin and ubiquitin-like proteases
Quantitative modeling of human diseases - methods and applications
Open questions on the mitochondrial unfolded protein response
Immunotherapy in myeloproliferative diseases
The evolutionary fate of mitochondrial aminoacyl-tRNA synthetases in amitochondrial organisms
Low level lysosomal membrane permeabilization for limited release and sub‐lethal functions of cathepsin proteases in the cytosol and nucleus
Mitochondrial machineries for protein import and assembly
Fatigue bei chronisch körperlichen Erkrankungen : = Fatigue in chronic physical diseases
Intramembrane proteases in neurodegenerative diseases
Autophagy in major human diseases
Mitochondrial protein import clogging as a mechanism of disease
Altered metabolic flexibility in inherited metabolic diseases of mitochondrial fatty acid metabolism
In the moonlight: non-catalytic functions of ubiquitin and ubiquitin-like proteases
Quantitative modeling of human diseases - methods and applications
Open questions on the mitochondrial unfolded protein response
Immunotherapy in myeloproliferative diseases
The evolutionary fate of mitochondrial aminoacyl-tRNA synthetases in amitochondrial organisms
Low level lysosomal membrane permeabilization for limited release and sub‐lethal functions of cathepsin proteases in the cytosol and nucleus
Mitochondrial machineries for protein import and assembly