Mitochondrial quality control: from molecule to organelle

Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease.

Mitochondria are essential for cellular bioenergetics by way of energy production in the form of ATP through the process of oxidative phosphorylation. This crucial task is executed by five multi-protein complexes of which mitochondrial NADH:ubiquinone oxidoreductase or complex I is the largest and most complicated one. During recent years, mutations in nuclear genes encoding structural subunits...

SnapShot: Mitochondrial Quality Control

Mitochondrial dynamics and mitochondrial quality control

Mitochondria are cellular energy powerhouses that play important roles in maintaining cell survival, cell death and cellular metabolic homeostasis. Timely removal of damaged mitochondria via autophagy (mitophagy) is thus critical for cellular homeostasis and function. Mitochondria are reticular organelles that have high plasticity for their dynamic structures and constantly undergo fission and ...

Quality control and organelle trafficking: ensuring functional organelles and cells

Mitochondrial quality control Jason Nielson (Rutter laboratory, University of Utah/Howard Hughes Medical Institute) described a protein quality control system centered on the Vms1-Cdc48 complex. This protein complex translocates to the mitochondrial outer membrane in response to various mitochondrial stresses, particularly those that elicit mitochondrial oxidant production. The search for the m...

Preserving organelle vitality: peroxisomal quality control mechanisms in yeast.

Cellular proteins and organelles such as peroxisomes are under continuous quality control. Upon synthesis in the cytosol, peroxisomal proteins are kept in an import-competent state by chaperones or specific proteins with an analogous function to prevent degradation by the ubiquitin-proteasome system. During protein translocation into the organelle, the peroxisomal targeting signal receptors (Pe...