Deep understanding of the pathophysiological role of the mitochondrial respiratory chain
(MRC) relies on a well-grounded model explaining how its biogenesis is regulated. The lack …

In eukaryotic cells, mitochondria perform cellular respiration through a series of redox
reactions ultimately reducing molecular oxygen to water. The system responsible for this …

The Commander complex is required for endosomal recycling of diverse transmembrane
cargos and is mutated in Ritscher-Schinzel syndrome. It comprises two sub-assemblies …

Pathological deterioration of mitochondrial function is increasingly linked with multiple
degenerative illnesses as a mediator of a wide range of neurologic and age-related chronic …

CLPB is a mitochondrial intermembrane space AAA+ domain–containing disaggregase.
CLPB mutations are associated with 3-methylglutaconic aciduria and neutropenia; however …

Abstract Human Tim8a and Tim8b are paralogous intermembrane space proteins of the
small TIM chaperone family. Yeast small TIMs function in the trafficking of proteins to the …

BRAWNIN was found as a mitochondrial respiratory complex III (CIII) assembly factor. Here,
we showed that the deletion rather than knockdown of BRAWNIN impaired the assembly of …

Metals are key elements for the survival and normal development of humans but can also be
toxic to cells when mishandled. In fact, even mild disruption of metal homeostasis causes a …

Background and objective Biallelic mutations in the COA7 gene have been associated with
spinocerebellar ataxia with axonal neuropathy type 3 (SCAN3), and a notable clinical …

Trimethyltin chloride (TMT) is a potent neurotoxin widely used as a constituent of polyvinyl
chloride plastic in the industrial and agricultural fields. However, the underlying mechanisms …