Subjects: MPP, Mitochondria processing peptidase, Molecular Biology, Ccs, Copper chaperone for SOD1, QSOX, Quiescin–sulfhydryl oxidase, CHCH, coiled coil–helix–coiled coil–helix, Thiol-disulfide exchange, GSH, reduced glutathione, GSSG, oxidized glutathione, HPO, hepatopoietin protein, Erv1, TIM, Translocase of the inner mitochondrial membrane, HSS, Hepatic regenerative stimulation substance, ALR, Augmenter of liver regeneration (also called GFER, HPO, HSS), MIA, Mitochondria import and assembly, COX, cytochrome C oxidase, SOD, Superoxide dismutase, TOM, Translocase of the outer mitochondrial membrane, Drp, Dynamin related GTPase protein, Review, SAM, sorting and assembly machinery, MISS, Mitochondrial intermembrane space sorting signal, NMR, Nuclear magnetic resonance, CytC, cytochrome C, Ero, Endoplasmic reticulum oxidation gene, CPC, Cysteine–Proline–Cysteine tripeptide, IMS, Intermembrane space, ER, Endoplasmic reticulum, Aim, Altered inheritance of mitochondria, Hot, Helper of Tim, PDI, Protein disulfide isomerase, Mitochondrial intermembrane space, Dsb, Disulfide bond formation genes, MICOS, Mitochondrial contact site and cristae organizing system, GFER, Growth factor, Erv1-like protein, HSP, Heat shock protein, Oxidative protein folding, Mia40, Cell Biology, Mitochondrial protein import, Erv, Essential for respiration and viability, CIA, cytosolic iron/sulphur cluster assembly, FAD, flavin adenine dinucleotide, ITS, Intermembrane space targeting signal
Mitochondria are fundamental organelles with a complex internal architecture that fulfill important diverse functions including iron–sulfur cluster assembly and cell respiration. Intense work for more than 30 years has identified the key protein import components and the pathways involved in protein targeting and assembly. More recently, oxidative folding has been discovered as one important mechanism for mitochondrial proteostasis whilst several human disorders have been linked to this pathway. We describe the molecular components of this pathway in view of their putative redox regulation and we summarize available evidence on the connections of these pathways to human disorders.