Cooperative assembly of the mitochondrial respiratory chain
E Fernández-Vizarra, C Ugalde - Trends in Biochemical Sciences, 2022 - cell.com
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 …
(MRC) relies on a well-grounded model explaining how its biogenesis is regulated. The lack …
Emerging mechanisms in the redox regulation of mitochondrial cytochrome c oxidase assembly and function
S Povea-Cabello, M Brischigliaro… - Biochemical Society …, 2024 - portlandpress.com
In eukaryotic cells, mitochondria perform cellular respiration through a series of redox
reactions ultimately reducing molecular oxygen to water. The system responsible for this …
reactions ultimately reducing molecular oxygen to water. The system responsible for this …
Structure of the endosomal Commander complex linked to Ritscher-Schinzel syndrome
MD Healy, KE McNally, R Butkovič, M Chilton, K Kato… - Cell, 2023 - cell.com
The Commander complex is required for endosomal recycling of diverse transmembrane
cargos and is mutated in Ritscher-Schinzel syndrome. It comprises two sub-assemblies …
cargos and is mutated in Ritscher-Schinzel syndrome. It comprises two sub-assemblies …
[HTML][HTML] Treatment and prevention of pathological mitochondrial dysfunction in retinal degeneration and in photoreceptor injury
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 …
degenerative illnesses as a mediator of a wide range of neurologic and age-related chronic …
CLPB disaggregase dysfunction impacts the functional integrity of the proteolytic SPY complex
CLPB is a mitochondrial intermembrane space AAA+ domain–containing disaggregase.
CLPB mutations are associated with 3-methylglutaconic aciduria and neutropenia; however …
CLPB mutations are associated with 3-methylglutaconic aciduria and neutropenia; however …
Human Tim8a, Tim8b and Tim13 are auxiliary assembly factors of mature Complex IV
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 …
small TIM chaperone family. Yeast small TIMs function in the trafficking of proteins to the …
Knockdown of BRAWNIN minimally affect mitochondrial complex III assembly in human cells
Y Wang, Y Shi, W Li, X Han, X Lin, D Liu, Y Lin… - … et Biophysica Acta (BBA …, 2024 - Elsevier
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 …
we showed that the deletion rather than knockdown of BRAWNIN impaired the assembly of …
Prime real estate: metals, cofactors and MICOS
AE Medlock, JC Hixon, T Bhuiyan… - Frontiers in Cell and …, 2022 - frontiersin.org
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 …
toxic to cells when mishandled. In fact, even mild disruption of metal homeostasis causes a …
Dystonia and Parkinsonism in COA7-related disorders: expanding the phenotypic spectrum
Y Higuchi, M Ando, F Kojima, J Yuan, A Hashiguchi… - Journal of …, 2024 - Springer
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 …
spinocerebellar ataxia with axonal neuropathy type 3 (SCAN3), and a notable clinical …
Quantitative proteomics reveals the neurotoxicity of trimethyltin chloride on mitochondria in the hippocampus of mice
Z Liu, L Wang, Y Wang, S Wu, C Peng, Y Wang… - NeuroToxicology, 2023 - Elsevier
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 …
chloride plastic in the industrial and agricultural fields. However, the underlying mechanisms …