As essential organelles, mitochondria are intimately integrated into the metabolism of a
eukaryotic cell. The maintenance of the functional integrity of the mitochondrial proteome …

Abstract The Mitochondrial Lon protease, also called LonP1 is a product of the nuclear gene
LONP1. Lon is a major regulator of mitochondrial metabolism and response to free radical …

Most mitochondrial precursor polypeptides are imported from the cytosol into the
mitochondrion, where they must efficiently undergo folding. Mitochondrial precursors are …

Human mitochondrial transcription factor A (TFAM) is a high-mobility group (HMG) protein at
the nexus of mitochondrial DNA (mtDNA) replication, transcription, and inheritance. Little is …

Lon is the major protease in the mitochondrial matrix in eukaryotes, and is well conserved
among species. Although a role for Lon in mitochondrial biogenesis has been proposed, the …

Cell survival depends on essential processes in mitochondria. Various proteases within
these organelles regulate mitochondrial biogenesis and ensure the complete degradation of …

CODAS syndrome is a multi-system developmental disorder characterized by cerebral,
ocular, dental, auricular, and skeletal anomalies. Using whole-exome and Sanger …

The elimination of oxidatively modified proteins is a crucial process in maintaining cellular
homeostasis, especially during stress. Mitochondria are protein-dense, high traffic …

The AAA+ Lon protease is a soluble single-ringed homo-oligomer, which represents the
most streamlined operational unit mediating ATP-dependent proteolysis. Despite its …

Maintenance of mitochondrial protein homeostasis is critical for proper cellular function.
Under normal conditions resident molecular chaperones and proteases maintain protein …