A preliminary study on the pathology and molecular mechanism of fumonisin B1 nephrotoxicity in young quails

J Li, M Zhu, R Xian, S Chen, Q Zang, H Zhu… - … Science and Pollution …, 2023 - Springer
Abstract Fumonisin B1 (FB1) is a widely present mycotoxin that accumulates in biological
systems and poses a health risk to animals. However, few studies have reported the …

Mitochondrial disease genes COA6, COX6B and SCO2 have overlapping roles in COX2 biogenesis

A Ghosh, AT Pratt, S Soma, SG Theriault… - Human molecular …, 2016 - academic.oup.com
Biogenesis of cytochrome c oxidase (CcO), the terminal enzyme of the mitochondrial
respiratory chain, is a complex process facilitated by several assembly factors. Pathogenic …

Loop recognition and copper-mediated disulfide reduction underpin metal site assembly of CuA in human cytochrome oxidase

MN Morgada, LA Abriata, C Cefaro… - Proceedings of the …, 2015 - National Acad Sciences
Maturation of cytochrome oxidases is a complex process requiring assembly of several
subunits and adequate uptake of the metal cofactors. Two orthologous Sco proteins (Sco1 …

A biallelic variant in COX18 cause isolated Complex IV deficiency associated with neonatal encephalo-cardio-myopathy and axonal sensory neuropathy

D Ronchi, M Garbellini, F Magri, F Menni… - European Journal of …, 2023 - nature.com
Pathogenic variants impacting upon assembly of mitochondrial respiratory chain Complex IV
(Cytochrome c Oxidase or COX) predominantly result in early onset mitochondrial disorders …

[HTML][HTML] COA6 facilitates cytochrome c oxidase biogenesis as thiol-reductase for copper metallochaperones in mitochondria

D Pacheu-Grau, M Wasilewski, S Oeljeklaus… - Journal of molecular …, 2020 - Elsevier
The mitochondrial cytochrome c oxidase, the terminal enzyme of the respiratory chain,
contains heme and copper centers for electron transfer. The conserved COX2 subunit …

Building the CuA site of cytochrome c oxidase: A complicated, redox-dependent process driven by a surprisingly large complement of accessory proteins

KA Jett, SC Leary - Journal of Biological Chemistry, 2018 - ASBMB
Cytochrome c oxidase (COX) was initially purified more than 70 years ago. A tremendous
amount of insight into its structure and function has since been gleaned from biochemical …

[HTML][HTML] Protein transduction domain-mediated delivery of recombinant proteins and in vitro transcribed mRNAs for protein replacement therapy of human severe …

AN Miliotou, PF Foltopoulou, A Ingendoh-Tsakmakidis… - Pharmaceutics, 2023 - mdpi.com
Mitochondrial disorders represent a heterogeneous group of genetic disorders with
variations in severity and clinical outcomes, mostly characterized by respiratory chain …

Mitochondrial respiration reduces exposure of the nucleus to oxygen

MP Mori, R Penjweini, J Ma, G Alspaugh… - Journal of Biological …, 2023 - ASBMB
The endosymbiotic theory posits that ancient eukaryotic cells engulfed O 2-consuming
prokaryotes, which protected them against O 2 toxicity. Previous studies have shown that …

High expression of COA6 is related to unfavorable prognosis and enhanced oxidative phosphorylation in lung adenocarcinoma

M Zhang, X Liao, G Ji, X Fan, Q Wu - International Journal of Molecular …, 2023 - mdpi.com
Mitochondrial metabolism plays an important role in the occurrence and development of
cancers. Cytochrome C oxidase assembly factor six (COA6) is essential in mitochondrial …

Regulation of COX Assembly and Function by Twin CX9C Proteins—Implications for Human Disease

S Gladyck, S Aras, M Hüttemann, LI Grossman - Cells, 2021 - mdpi.com
Oxidative phosphorylation is a tightly regulated process in mammals that takes place in and
across the inner mitochondrial membrane and consists of the electron transport chain and …