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Bioenergetics dysfunction, mitochondrial permeability transition pore opening and lipid peroxidation induced by hydrogen sulfide as relevant pathomechanisms underlying the neurological dysfunction characteristic of ethylmalonic encephalopathy.
Cardoso GMF, Pletsch JT, Parmeggiani B, Grings M, Glanzel NM, Bobermin LD, Amaral AU, Wajner M, Leipnitz G. Cardoso GMF, et al. Among authors: glanzel nm. Biochim Biophys Acta Mol Basis Dis. 2017 Sep;1863(9):2192-2201. doi: 10.1016/j.bbadis.2017.06.007. Epub 2017 Jun 15. Biochim Biophys Acta Mol Basis Dis. 2017. PMID: 28624490 Free article.
Evidence that thiol group modification and reactive oxygen species are involved in hydrogen sulfide-induced mitochondrial permeability transition pore opening in rat cerebellum.
de Moura Alvorcem L, Britto R, Parmeggiani B, Glanzel NM, da Rosa-Junior NT, Cecatto C, Bobermin LD, Amaral AU, Wajner M, Leipnitz G. de Moura Alvorcem L, et al. Among authors: glanzel nm. Mitochondrion. 2019 Jul;47:141-150. doi: 10.1016/j.mito.2018.11.001. Epub 2018 Nov 3. Mitochondrion. 2019. PMID: 30399433
The mitochondrial-targeted reactive species scavenger JP4-039 prevents sulfite-induced alterations in antioxidant defenses, energy transfer, and cell death signaling in striatum of rats.
Glänzel NM, Grings M, da Rosa-Junior NT, de Carvalho LMC, Mohsen AW, Wipf P, Wajner M, Vockley J, Leipnitz G. Glänzel NM, et al. J Inherit Metab Dis. 2021 Mar;44(2):481-491. doi: 10.1002/jimd.12310. Epub 2020 Sep 14. J Inherit Metab Dis. 2021. PMID: 32882059 Free PMC article.
Antioxidant system disturbances and mitochondrial dysfunction induced by 3-methyglutaric acid in rat heart are prevented by bezafibrate.
da Rosa-Junior NT, Parmeggiani B, Glänzel NM, de Moura Alvorcem L, Brondani M, Britto R, Grings M, Ortiz VD, Turck P, da Rosa Araujo AS, Wajner M, Leipnitz G. da Rosa-Junior NT, et al. Among authors: glanzel nm. Eur J Pharmacol. 2022 Jun 5;924:174950. doi: 10.1016/j.ejphar.2022.174950. Epub 2022 Apr 14. Eur J Pharmacol. 2022. PMID: 35430210
Sulfite Impairs Bioenergetics and Redox Status in Neonatal Rat Brain: Insights into the Early Neuropathophysiology of Isolated Sulfite Oxidase and Molybdenum Cofactor Deficiencies.
Pramio J, Grings M, da Rosa AG, Ribeiro RT, Glanzel NM, Signori MF, Marcuzzo MB, Bobermin LD, Wyse ATS, Quincozes-Santos A, Wajner M, Leipnitz G. Pramio J, et al. Among authors: glanzel nm. Cell Mol Neurobiol. 2023 Aug;43(6):2895-2907. doi: 10.1007/s10571-023-01328-6. Epub 2023 Mar 2. Cell Mol Neurobiol. 2023. PMID: 36862242
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