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Title[redir]:
Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency - PMC
Description:
The gene encoding the Krebs cycle enzyme fumarate hydratase (FH) is mutated in hereditary leiomyomatosis and renal cell cancer (HLRCC). Loss of FH activity causes accumulation of intracellular fumarate, which can directly modify cysteine residues to ...
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Keywords {🔍}
doi, succination, aco, fumarate, protein, fhko, mefs, cell, google, scholar, aconitase, pubmed, mitochondrial, activity, cells, figure, cysteine, residues, proteins, pmc, article, succinated, fragment, ion, mass, free, targets, cycle, performed, fhdeficient, data, tissue, adam, cancer, metabolism, analyses, fhwt, citrate, krebs, dehydrogenase, isoform, oflaherty, cellular, previously, frizzell, soga, cluster, mitochondria, peptide, analysis,
Topics {✒️}
isoforms β\δ\ε\γ fh1 fl/fl ksp-cre iron-sulfur-binding cysteine residues iron-sulfur cluster assembly dose-dependent fumarate-mediated succination iron-sulfur cluster binding supplemental information mmc1 performed lc-ms/ms analyses thioredoxin-dependent peroxide reductases cdgsh iron-sulfur domain oncogenic hif-dependent pathways c-terminal fragment ion n-terminal fragment ion thioredoxin-dependent peroxide reductase pef1/v5-hisa vector creatine kinase u-type pmc beta search δ-1-pyrroline-5-carboxylate synthase lc-ms/ms analyses open-access article distributed bifunctional aminoacyl-trna synthetase fumarate-mediated succination occurs monofunctional c1-tetrahydrofolate synthase 2og-dependent oxygenases similar [google scholar] schneider egg jelly-related protein rna-binding protein raly repeated ms/ms analysis iron-binding cysteine residues lc-ms/ms analysis s-methyl-5′-thioadenosine phosphorylase histone-binding protein rbbp7 extended experimental procedures deuterium-labeled [d5]glutamine probability-based protein identification scaffold attachment factor license information pmcid free sulfhydryl groups diverse cellular pathways iron response element fh-deficient cells leads fumarate hydratase-deficient cells glutamine-dependent reductive mechanism references adam fumarate hydratase-deficient mice full ms scan v5-tagged aco2 gene label-free quantitation atomic force microscopy trypsin-digested aco2 derived
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