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163 results

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Quoted phrase not found in phrase index: "Succinate-coenzyme Q reductase deficiency"
Page 1
SDHA/B reduction promotes hepatocellular carcinoma by facilitating the deNEDDylation of cullin1 and stabilizing YAP/TAZ.
Yuan T, Zhou T, Qian M, Du J, Liu Y, Wang J, Li Y, Fan G, Yan F, Dai X, Li X, Wu Y, Dong X, He Q, Zhu H, Yang B. Yuan T, et al. Hepatology. 2023 Jul 1;78(1):103-119. doi: 10.1002/hep.32621. Epub 2022 Jul 6. Hepatology. 2023. PMID: 35713976
However, the underlying mechanisms of how impaired SDH activity promotes HCC remain unclear. APPROACH AND RESULTS: In this study, we observed remarkable downregulations of SDH subunits A and B (SDHA/B) in chronic liver injury-induced murine HCC models and patient samples. …
However, the underlying mechanisms of how impaired SDH activity promotes HCC remain unclear. APPROACH AND RESULTS: In this study, we obse …
Succinate Dehydrogenase Complex: An Updated Review.
Rasheed MRHA, Tarjan G. Rasheed MRHA, et al. Arch Pathol Lab Med. 2018 Dec;142(12):1564-1570. doi: 10.5858/arpa.2017-0285-RS. Epub 2018 Oct 5. Arch Pathol Lab Med. 2018. PMID: 30289269 Free article. Review.
Remarkable discoveries in the last 17 years have led to the delineation of the SDH complex deficiency syndrome and its multiple pathogenic branches. Here we provide an updated overview of SDH deficiency in order to raise awareness of its multiple connotations …
Remarkable discoveries in the last 17 years have led to the delineation of the SDH complex deficiency syndrome and its multipl …
Succinate dehydrogenase (SDH)-deficient neoplasia.
Gill AJ. Gill AJ. Histopathology. 2018 Jan;72(1):106-116. doi: 10.1111/his.13277. Histopathology. 2018. PMID: 29239034 Review.
The succinate dehydrogenase (SDH) complex is a key respiratory enzyme composed of four subunits: SDHA, SDHB, SDHC and SDHD. ...It is particularly associated with SDHB mutation, although SDHC and SDHA mutation occur. SDH-deficient pituitary adenomas are recognized, b …
The succinate dehydrogenase (SDH) complex is a key respiratory enzyme composed of four subunits: SDHA, SDHB, SDHC and SDHD. ...It is …
Pathological Features in Paediatric Patients with TK2 Deficiency.
Jou C, Nascimento A, Codina A, Montoya J, López-Gallardo E, Emperador S, Ruiz-Pesini E, Montero R, Natera-de Benito D, Ortez CI, Marquez J, Zelaya MV, Gutierrez-Mata A, Badosa C, Carrera-García L, Expósito-Escudero J, Roldán M, Camara Y, Marti R, Ferrer I, Jimenez-Mallebrera C, Artuch R. Jou C, et al. Int J Mol Sci. 2022 Sep 20;23(19):11002. doi: 10.3390/ijms231911002. Int J Mol Sci. 2022. PMID: 36232299 Free PMC article.
Thymidine kinase (TK2) deficiency causes mitochondrial DNA depletion syndrome. We aimed to report the clinical, biochemical, genetic, histopathological, and ultrastructural features of a cohort of paediatric patients with TK2 deficiency. ...All muscle biopsies prese …
Thymidine kinase (TK2) deficiency causes mitochondrial DNA depletion syndrome. We aimed to report the clinical, biochemical, genetic, …
Interplay of Impaired Cellular Bioenergetics and Autophagy in PMM2-CDG.
Ligezka AN, Budhraja R, Nishiyama Y, Fiesel FC, Preston G, Edmondson A, Ranatunga W, Van Hove JLK, Watzlawik JO, Springer W, Pandey A, Morava E, Kozicz T. Ligezka AN, et al. Genes (Basel). 2023 Aug 4;14(8):1585. doi: 10.3390/genes14081585. Genes (Basel). 2023. PMID: 37628636 Free PMC article.
This dysfunction was evidenced by decreased mitochondrial maximal and ATP-linked respiration, as well as decreased complex I function of the mitochondrial electron transport chain. ...Interestingly, serum sorbitol levels (a biomarker of disease severity) and …
This dysfunction was evidenced by decreased mitochondrial maximal and ATP-linked respiration, as well as decreased complex I function …
Succinate dehydrogenase deficient gastrointestinal stromal tumors (GISTs) - a review.
Miettinen M, Lasota J. Miettinen M, et al. Int J Biochem Cell Biol. 2014 Aug;53:514-9. doi: 10.1016/j.biocel.2014.05.033. Epub 2014 Jun 2. Int J Biochem Cell Biol. 2014. PMID: 24886695 Free PMC article. Review.
Loss of function of the succinate dehydrogenase complex characterizes a rare group of human tumors including some gastrointestinal stromal tumors, paragangliomas, renal carcinomas, and pituitary adenomas, and these can all be characterized as SDH-deficient tumors. . …
Loss of function of the succinate dehydrogenase complex characterizes a rare group of human tumors including some gastrointestinal st …
Succinate dehydrogenase (SDH) and mitochondrial driven neoplasia.
Gill AJ. Gill AJ. Pathology. 2012 Jun;44(4):285-92. doi: 10.1097/PAT.0b013e3283539932. Pathology. 2012. PMID: 22544211 Review.
The proteins are assembled in the mitochondria to form the mitochondrial complex 2, a key respiratory enzyme which links the Krebs cycle and the electron transport chain. ...PHEO/PGL associated with SDHA mutation also show negative staining for SDHA as well a …
The proteins are assembled in the mitochondria to form the mitochondrial complex 2, a key respiratory enzyme which links the Krebs cy …
Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain.
Doni D, Cavion F, Bortolus M, Baschiera E, Muccioli S, Tombesi G, d'Ettorre F, Ottaviani D, Marchesan E, Leanza L, Greggio E, Ziviani E, Russo A, Bellin M, Sartori G, Carbonera D, Salviati L, Costantini P. Doni D, et al. Cell Death Dis. 2023 Dec 8;14(12):805. doi: 10.1038/s41419-023-06320-y. Cell Death Dis. 2023. PMID: 38062036 Free PMC article.
Frataxin participates in the mitochondrial assembly of FeS clusters, redox cofactors of the respiratory complexes I, II and III. To date it is still unclear how frataxin deficiency culminates in the decrease of bioenergetics efficiency in FRDA patients' cells. ...Fu …
Frataxin participates in the mitochondrial assembly of FeS clusters, redox cofactors of the respiratory complexes I, II and III. To d …
SDHi fungicides: An example of mitotoxic pesticides targeting the succinate dehydrogenase complex.
Duarte Hospital C, Tête A, Debizet K, Imler J, Tomkiewicz-Raulet C, Blanc EB, Barouki R, Coumoul X, Bortoli S. Duarte Hospital C, et al. Environ Int. 2023 Oct;180:108219. doi: 10.1016/j.envint.2023.108219. Epub 2023 Sep 19. Environ Int. 2023. PMID: 37778286 Free article. Review.
The SDH is involved in two interconnected metabolic processes for energy production: the transfer of electrons in the mitochondrial respiratory chain and the oxidation of succinate to fumarate in the Krebs cycle. In humans, inherited SDH deficiencies may cause major pathol …
The SDH is involved in two interconnected metabolic processes for energy production: the transfer of electrons in the mitochondrial respirat …
Gastrointestinal stromal tumours: from KIT to succinate dehydrogenase.
Doyle LA, Hornick JL. Doyle LA, et al. Histopathology. 2014 Jan;64(1):53-67. doi: 10.1111/his.12302. Epub 2013 Nov 28. Histopathology. 2014. PMID: 24117705 Review.
The discovery of activating mutations in the tyrosine kinase receptor genes KIT and PDGFRA has led to the development of effective targeted therapies for gastrointestinal stromal tumours (GISTs). Specific genotypes, in part, predict the response to treatment with tyrosine …
The discovery of activating mutations in the tyrosine kinase receptor genes KIT and PDGFRA has led to the development of effective targeted …
163 results