Stop wasting protein—Proteasome inhibition to target diseases linked to mitochondrial import

Mitochondrial dysfunction is linked to various human diseases. Symptoms can occur early in life or manifest progressively during life and include poor muscle coordination or weakness, neurological or developmental problems, or immunodeficiency (Lightowlers et al, 2015). Most mitochondrial diseases are caused by mutations in genes encoding mitochondrial proteins. Mutations can affect protein functions in many ways; they can not only impair enzymatic activities, but also lower protein stability, hamper assembly into multimeric protein complexes, or abrogate protein transport into mitochondria. Understanding the impact of mutations on protein function is crucial to understand pathophysiological mechanisms of mitochondrial diseases and to develop therapeutic approaches.

Object Details

• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch

References and Relationships

• Bibliographic citation: Stop wasting protein—Proteasome inhibition to target diseases linked to mitochondrial import ; volume:11 ; number:5 ; year:2019 ; extent:3 ; EMBO molecular medicine / European Molecular Biology Organization ; 11, Heft 5 (2019) (gesamt 3)

Contributors, Places and Time

• Creator: Habich, Markus ; Riemer, Jan

Further information

• DOI: 10.15252/emmm.201910441
• URN: urn:nbn:de:101:1-2022080808123759000446
• Rights: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 15.08.2025, 7:29 AM CEST