دورية أكاديمية
Disruption of Bioenergetics in the Intestine of Wistar Rats Caused by Hydrogen Sulfide and Thiosulfate: A Potential Mechanism of Chronic Hemorrhagic Diarrhea in Ethylmalonic Encephalopathy.
| العنوان: | Disruption of Bioenergetics in the Intestine of Wistar Rats Caused by Hydrogen Sulfide and Thiosulfate: A Potential Mechanism of Chronic Hemorrhagic Diarrhea in Ethylmalonic Encephalopathy. |
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| المؤلفون: | Frusciante MR; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil., Signori MF; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil., Parmeggiani B; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil., Grings M; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil., Pramio J; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil., Cecatto C; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil., de Andrade Silveira J; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil., Aubin MR; Programa de Pós-Graduação em Fisiologia, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 500 Sarmento Leite Street, Porto Alegre, RS, 90035-190, Brazil.; Laboratório de Células, Tecidos e Genes - Centro de Pesquisa Experimental, HCPA, Porto Alegre, RS, Brazil., Santos LA; Programa de Pós-Graduação em Fisiologia, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 500 Sarmento Leite Street, Porto Alegre, RS, 90035-190, Brazil.; Laboratório de Células, Tecidos e Genes - Centro de Pesquisa Experimental, HCPA, Porto Alegre, RS, Brazil., Paz AH; Programa de Pós-Graduação em Fisiologia, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 500 Sarmento Leite Street, Porto Alegre, RS, 90035-190, Brazil.; Laboratório de Células, Tecidos e Genes - Centro de Pesquisa Experimental, HCPA, Porto Alegre, RS, Brazil., Wajner M; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil.; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil.; Serviço de Genética Médica, Hospital de Clínicas de Porto Alegre, 2350 Ramiro Barcelos Street, Porto Alegre, RS, 90035-903, Brazil., Leipnitz G; Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil. guilhian@ufrgs.br.; Programa de Pós-Graduação em Fisiologia, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 500 Sarmento Leite Street, Porto Alegre, RS, 90035-190, Brazil. guilhian@ufrgs.br.; Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, 2600 Ramiro Barcelos Street-Attached, Porto Alegre, RS, 90035-003, Brazil. guilhian@ufrgs.br. |
| المصدر: | Cell biochemistry and biophysics [Cell Biochem Biophys] 2023 Dec; Vol. 81 (4), pp. 683-695. Date of Electronic Publication: 2023 Aug 17. |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Humana Press Country of Publication: United States NLM ID: 9701934 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1559-0283 (Electronic) Linking ISSN: 10859195 NLM ISO Abbreviation: Cell Biochem Biophys Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Totowa, NJ : Humana Press, c1996- |
| مواضيع طبية MeSH: | Hydrogen Sulfide*, Humans ; Rats ; Animals ; Rats, Wistar ; Thiosulfates/pharmacology ; Caco-2 Cells ; Energy Metabolism ; Sulfides ; Intestines ; Diarrhea ; Protein Isoforms/metabolism |
| مستخلص: | Ethylmalonic encephalopathy (EE) is a severe inherited metabolic disorder that causes tissue accumulation of hydrogen sulfide (sulfide) and thiosulfate in patients. Although symptoms are predominantly neurological, chronic hemorrhagic diarrhea associated with intestinal mucosa abnormalities is also commonly observed. Considering that the pathophysiology of intestinal alterations in EE is virtually unknown and that sulfide and thiosulfate are highly reactive molecules, the effects of these metabolites were investigated on bioenergetic production and transfer in the intestine of rats. We observed that sulfide reduced NADH- and FADH (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.) |
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| معلومات مُعتمدة: | # 402440/2021-8 Conselho Nacional de Desenvolvimento Científico e Tecnológico; # 16/2551-0000465-0 Fundação de Amparo à Pesquisa do Estado do Rio Grande do Sul; # 465671/2014-4 Instituto Nacional de Ciência e Tecnologia em Excitotoxicidade e Neuroproteção |
| فهرسة مساهمة: | Keywords: Creatine kinase; Ethylmalonic encephalopathy; Hydrogen sulfide; Intestine; Mitochondrial respiration; Thiosulfate |
| المشرفين على المادة: | YY9FVM7NSN (Hydrogen Sulfide) 0 (Thiosulfates) 0 (Sulfides) 0 (Protein Isoforms) |
| SCR Disease Name: | Ethylmalonic encephalopathy |
| تواريخ الأحداث: | Date Created: 20230817 Date Completed: 20231030 Latest Revision: 20231030 |
| رمز التحديث: | 20231215 |
| DOI: | 10.1007/s12013-023-01161-0 |
| PMID: | 37589888 |
| قاعدة البيانات: | MEDLINE |
Find this article in full text from Springer Verlag. 
Full Text Finder | تدمد: | 1559-0283 |
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| DOI: | 10.1007/s12013-023-01161-0 |