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Showing 1 to 14 of 14 for “"propionyl-CoA"”.
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Studies on the mechanism of action of propionyl-CoA carboxylase
Propionyl-CoA carboxylase has been purified to a state of near nomogeniety, and some of its enzymatic properties relating to substrate binding and mechanism of action have been studied. The enzyme was not found to catalyze the incorporation of solvent tritium at the c-carbon of propionylâ CoA in …
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Studies on some enzymatic properties of mitochondrial propionyl carboxylase
Propionyl carboxylase purified from bovine liver mitochondria catalyzes the carboxylation of 992 micromoles of propionyl-CoA per hour per milligram of protein. Relative carboxylation rates for acetyl-, propionyl-, butyryl-, and valeryl-CoA remain constant during purification. The carboxylase is …
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Roles of the Methylcitrate and Methylmalonyl-COA Pathways in Mycobacterial Metabolism and Pathogenesis
… carbon sources in vitro and can all generate propionyl-CoA. The methylcitrate cycle, which, in M. tuberculosis, uses a bifunctional isocitrate lyase/methylisocitrate lyase (ICL/MCL), is one of the two routes for metabolism of propionyl-CoA. A mutant strain of M. tuberculosis lacking the …
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Propionyl holocarboxylase synthesis
… rats, catalyzes the ATP-dependent synthesis of propionyl holocarboxylase from d-biotin and propionyl apocarboxylase. This system has been resolved by alumina C<sub>∂</sub> gel fractionation into two essential components; (a) gel supernatant which contains propionyl apocarboxylase and (b) gel …
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Structural and Functional Studies of Biotin-Dependent Carboxylases
… We engineer a novel chimeric variant of propionyl-CoA carboxylase (PCC) and produce the first crystal structure of its 750-kDa α6β6 holoenzyme. This structure reveals the architecture of PCC's twelve catalytic domains and allows the mapping of its disease-associated gene mutations to …
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Generation of a Medaka Fish Model of Propionic Acidemia for Development of Novel Therapies.
… caused by deficiency of the mitochondrial enzyme propionyl-CoA carboxylase (PCC). The disease presents with acute, recurrent and life-threatening crises of metabolic decompensation starting from the newborn period. Patients also suffer from multi-organ complications, neurological dysfunction, and …
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Studies on Methylmalonyl-CoA Mutase from Escherichia coli
Methylmalonyl-CoA mutase (MCM, E.C. 5.4.99.2), a coenzyme B12-dependent enzyme, catalyses the inter conversion of succinyl-CoA and methylmalonyl-CoA. The gene (sbm) encoding this enzyme is found in Escherichia coli (E. coli) at 62.3min on the E. coli chromosome. However, the metabolic role of this …
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Molekulare Mechanismen der Aldosteron-Wirkung und Interaktionen des Mineralocorticoid-Rezeptors in Nierenzellen
… im MR-Komplex gefunden wurden. GRP78 und -Propionyl-CoA-carboxylase Vorläufermolekül wurden beim unstimulierten MR wie auch nach einer Stimulation mit Aldosteron isoliert, jedoch nicht mehr nach einer Inkubation mit Spironolacton. Diese Befunde könnten auf eine bisher unerkannte Komplexität …
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Autism Spectrum Disorder and Mitochondrial Dysfunction: The Role of Mitochondrial Dynamics
… methylation and metabolomics studies. Propionyl-CoA Carboxylase Subunit Beta (PCCB) was differentially methylated in our cohort ASD study, and its dysregulation can lead to the accumulation of propionic acid. This links PCCB's function to a well-established animal model that uses sodium …
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Autotrophe CO2-Fixierung in thermophilen Mikroorganismen
… reduktive Citrat-Zyklus, der reduktive Acetyl-CoA-Weg und der 3-Hydroxypropionat-Zyklus. Letzterer wurde bisher nur im phototrophen Bakterium Chloroflexus aurantiacus nachgewiesen. <br>In den letzten Jahren wurden zahlreiche thermophile und hyperthermophile Vertreter der Crenarchaeota isoliert, …
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Mitochondrial mechanisms in autism spectrum disorder: characterizing the neurotoxic effects of propionic acid in vitro
… converging on mitochondrial metabolism and Propionyl CoA Carboxylase B (PCCB). This project aimed to better characterise the mitochondrial component of ASD aetiology using an in vitro system to mechanistically interrogate the molecular signatures found in an understudied SA population. …
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THE EFFECT OF DIETARY FIBRE ON HUMAN LIPID METABOLISM
… produced from the fermentation in the colon. Propionyl-CoA may compete with acetyl-CoA for binding sites on HMG-CoA synthase. Changes in serum glucagon have been reported when SCFA are infused rectally, and propionate is known to have a high affinity for carnitine. Increased glucagon secretion …
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Structural and mechanistic investigation towards the regulation of M.tb isocitrate lyase
… by lipid metabolite acetyl-coenzyme A (acetyl-CoA) and its structural analogues. The interactions between host and pathogen are important for M. tb as the bacteria may reside inside its human host for decades. Human macrophages produce itaconate as an antimicrobial metabolite. Previous studies …
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In vitro investigation of the molecular relationship between mitochondrial dysfunction and neurogenesis in autism aetiology
… A key differentially methylated gene was Propionyl-CoA Carboxylase Subunit B (PCCB), a mitochondrial enzyme involved in metabolizing short fatty acids like propionic acid (PPA). Disruption of PCCB function could result in toxic accumulation of PPA. Furthermore, PPA is used to induce …