| CHCHD4 regulates the expression of mitochondrial genes that are essential for tumour cell growth. | CHCHD4 regulates the expression of mitochondrial genes that are essential for tumour cell growth.
Thomas LW, Stephen JM, Ashcroft M. | 08/22/2024 |
| [The glutathionylation of the human mitochondrial protein MIA40 regulates ROS homeostasis]. | [The glutathionylation of the human mitochondrial protein MIA40 regulates ROS homeostasis].
Arab R, Sitolle J, Ziga J, Golinelli-Cohen MP. | 02/28/2024 |
| Impaired AIF-CHCHD4 interaction and mitochondrial calcium overload contribute to auditory neuropathy spectrum disorder in patient-iPSC-derived neurons with AIFM1 variant. | Impaired AIF-CHCHD4 interaction and mitochondrial calcium overload contribute to auditory neuropathy spectrum disorder in patient-iPSC-derived neurons with AIFM1 variant.
Qiu Y, Wang H, Fan M, Pan H, Guan J, Jiang Y, Jia Z, Wu K, Zhou H, Zhuang Q, Lei Z, Ding X, Cai H, Dong Y, Yan L, Lin A, Fu Y, Zhang D, Yan Q, Wang Q., Free PMC Article | 07/3/2023 |
| CHCHD4 (MIA40) and the mitochondrial disulfide relay system. | CHCHD4 (MIA40) and the mitochondrial disulfide relay system.
Al-Habib H, Ashcroft M., Free PMC Article | 01/29/2022 |
| AIF meets the CHCHD4/Mia40-dependent mitochondrial import pathway. | AIF meets the CHCHD4/Mia40-dependent mitochondrial import pathway.
Reinhardt C, Arena G, Nedara K, Edwards R, Brenner C, Tokatlidis K, Modjtahedi N. | 10/24/2020 |
| First group to discover the expression of the human CHCHD4 isoforms and first to call the human gene CHCHD4, and to clone and show the expression of its alternatively expressed CHCHD4 isoforms (CHCHD4.1 and CHCHD4.2). | Human CHCHD4 mitochondrial proteins regulate cellular oxygen consumption rate and metabolism and provide a critical role in hypoxia signaling and tumor progression.
Yang J, Staples O, Thomas LW, Briston T, Robson M, Poon E, Simões ML, El-Emir E, Buffa FM, Ahmed A, Annear NP, Shukla D, Pedley BR, Maxwell PH, Harris AL, Ashcroft M., Free PMC Article | 04/19/2017 |
| Compared to wild type control littermates, mice with a knockout of CHCHD4 exhibit reduced weight gain when fed a high-fat diet. | Metabolic epistasis among apoptosis-inducing factor and the mitochondrial import factor CHCHD4.
Modjtahedi N, Hangen E, Gonin P, Kroemer G., Free PMC Article | 06/28/2016 |
| Data show that the redox state of cytochrome c oxidase assembly protein 17 (Cox17), mitochondrial membrane transport protein Mia40 and superoxide dismutase 1 (SOD1) in the cytoplasm were directly observed with in-cell NMR spectroscopy. | Direct structural evidence of protein redox regulation obtained by in-cell NMR.
Mercatelli E, Barbieri L, Luchinat E, Banci L. | 05/28/2016 |
| our findings suggest that MIA40 reduction contributes to the effects of AIF deficiency on OXPHOS, as it may impact on the correct assembly and maintenance of the respiratory subunits. | Loss of apoptosis-inducing factor critically affects MIA40 function.
Meyer K, Buettner S, Ghezzi D, Zeviani M, Bano D, Nicotera P., Free PMC Article | 05/14/2016 |
| results demonstrate an indispensable role for hMIA40 for the export of Fe-S clusters from mitochondria. | Human mitochondrial MIA40 (CHCHD4) is a component of the Fe-S cluster export machinery.
Murari A, Thiriveedi VR, Mohammad F, Vengaldas V, Gorla M, Tammineni P, Krishnamoorthy T, Sepuri NB. | 01/2/2016 |
| Data indicate that poptosis-inducing factor (AIF) controls the mitochondrial import of mitochondrial membrane transport protein CHCHD4. | Interaction between AIF and CHCHD4 Regulates Respiratory Chain Biogenesis.
Hangen E, Féraud O, Lachkar S, Mou H, Doti N, Fimia GM, Lam NV, Zhu C, Godin I, Muller K, Chatzi A, Nuebel E, Ciccosanti F, Flamant S, Bénit P, Perfettini JL, Sauvat A, Bennaceur-Griscelli A, Ser-Le Roux K, Gonin P, Tokatlidis K, Rustin P, Piacentini M, Ruvo M, Blomgren K, Kroemer G, Modjtahedi N. | 09/26/2015 |
| In aggregate these data suggest that the Mia40/lfALR system has a broad sequence specificity and that potential substrates may be protected from adventitious oxidation by kinetic sequestration within the mitochondrial IMS. | Mia40 is a facile oxidant of unfolded reduced proteins but shows minimal isomerase activity.
Hudson DA, Thorpe C., Free PMC Article | 09/26/2015 |
| Import and oxidative folding of proteins are kinetically and functionally coupled and depend on the expression of Mia40, ALR, and the intracellular glutathione pool. | Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells.
Fischer M, Horn S, Belkacemi A, Kojer K, Petrungaro C, Habich M, Ali M, Küttner V, Bien M, Kauff F, Dengjel J, Herrmann JM, Riemer J., Free PMC Article | 02/8/2014 |
| Data indicate that decreased CHCHD4 expression prevents the mitochondrial translocation of p53 while augmenting its nuclear localization and activity. | Mitochondrial disulfide relay mediates translocation of p53 and partitions its subcellular activity.
Zhuang J, Wang PY, Huang X, Chen X, Kang JG, Hwang PM., Free PMC Article | 01/4/2014 |
| illustrate a very atypical behaviour for the Mia40 precursor compared to other substrates of the MIA pathway. By contrast, interaction with Erv1 occurs after 5 min of import and relies on a more stringent specificity | Biogenesis of yeast Mia40 - uncoupling folding from import and atypical recognition features.
Chatzi A, Sideris DP, Katrakili N, Pozidis C, Tokatlidis K. | 11/23/2013 |
| mitochondrial localization of MIA40 requires sulfhydryl oxidase ALR in heterologous expression yeast system. | Disulfide bond formation: sulfhydryl oxidase ALR controls mitochondrial biogenesis of human MIA40.
Sztolsztener ME, Brewinska A, Guiard B, Chacinska A. | 07/27/2013 |
| Molecular recognition and substrate mimicry drive the electron-transfer process between MIA40 and ALR. | Molecular recognition and substrate mimicry drive the electron-transfer process between MIA40 and ALR.
Banci L, Bertini I, Calderone V, Cefaro C, Ciofi-Baffoni S, Gallo A, Kallergi E, Lionaki E, Pozidis C, Tokatlidis K., Free PMC Article | 05/28/2011 |
| Observational study of gene-disease association. (HuGE Navigator) | Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.
Hendrickson SL, Lautenberger JA, Chinn LW, Malasky M, Sezgin E, Kingsley LA, Goedert JJ, Kirk GD, Gomperts ED, Buchbinder SP, Troyer JL, O'Brien SJ., Free PMC Article | 12/5/2010 |
| Catalysis involves a flow of reducing equivalents from the reduced CxC cysteine motif of Mia40 to distal and then proximal CxxC motifs of long-form ALR to the flavin ring and, finally, to cytochrome c or molecular oxygen. | Augmenter of liver regeneration: substrate specificity of a flavin-dependent oxidoreductase from the mitochondrial intermembrane space.
Daithankar VN, Farrell SR, Thorpe C., Free PMC Article | 01/21/2010 |
| analysis of how mitochondrial biogenesis switches the sorting pathway of the intermembrane space receptor Mia40 | Mitochondrial biogenesis, switching the sorting pathway of the intermembrane space receptor Mia40.
Chacinska A, Guiard B, Müller JM, Schulze-Specking A, Gabriel K, Kutik S, Pfanner N., Free PMC Article | 01/21/2010 |
| After passage across the translocase of the mitochondrial outer membrane Erv1 interacts via disulfide bonds with Mia40. | The sulfhydryl oxidase Erv1 is a substrate of the Mia40-dependent protein translocation pathway.
Terziyska N, Grumbt B, Bien M, Neupert W, Herrmann JM, Hell K. | 01/21/2010 |
| biogenesis and function of MIA40 in the mitochondrial intermembrane space is dependent on redox processes involving conserved cysteine residues | Functional and mutational characterization of human MIA40 acting during import into the mitochondrial intermembrane space.
Hofmann S, Rothbauer U, Mühlenbein N, Baiker K, Hell K, Bauer MF. | 01/21/2010 |