Cystathionine beta-synBiosynthesisHomocysteine metabolismEnzymeSerineGlutamine SynthaseOxidativePathwaysProteinsDeficiencyPathwayNitricProteinTrans-sulfurationLipid metabolismUric acidMethanethiolDerived from methionineMitochondriaGeneticAcceptorReactionHepaticSulfurVitaminFolateBiochemical processesTypicallyLipidsMechanismGlucoseMetabolicLysineSecondaryRoleBoneRolesFormKidneyDeterminesBody
Cystathionine beta-syn5
- The most common block in the homocysteine pathway is the conversion of cystathionine to cysteine, which requires vitamin B6 to activate the cystathionine beta-synthase enzyme. (drprincetta.com)
- It is caused by the deficiency of the enzyme cystathionine beta synthase, and the deficiency of folic acid, vitamin B 12 and pyridoxine (vitamin B 6 ), or mutations of related enzymes. (mhmedical.com)
- Classical homocystinuria (HCU) is a rare genetic metabolic disorder caused by a deficiency in the enzyme cystathionine beta synthase (CBS). (travere.com)
- In that article I discussed how there appears to be two differing perspectives on the nature of CBS (cystathionine beta synthase) gene mutations: the literature's contention of "down-regulated" CBS enzyme activity among carriers of certain mutations, and certain empirical, clinical observations of the opposite situation, specifically that certain CBS gene mutations can cause an "up-regulation" to enzyme activity. (metabolichealing.com)
- This article is a follow-up, based upon my own current hypotheses as well as literature-cited observations regarding these complex yet seemingly critical discussions regarding CBS (cystathionine beta synthase) and the transsulfuration pathway. (metabolichealing.com)
Biosynthesis2
- More specifically, CBS activates the sulfuration process, the biosynthesis of the sulfur-containing amino acid cysteine, and the fate of residual sulfur byproducts through various downstream processes. (metabolichealing.com)
- Reconstructed carbon and sulfur metabolic pathways, coupled with RNA-Seq analysis, suggested a marginal Crabtree effect under high glucose and activation of sulfur metabolism toward methionine biosynthesis under sulfur limitation in this yeast. (biomedcentral.com)
Homocysteine metabolism7
- This study was aimed at investigating the effects of quercetin on mRNA expression and activity of critical enzymes in homocysteine metabolism in rats fed a methionine-enriched diet. (hindawi.com)
- N-Acetyl-cysteine supports homocysteine metabolism by mobilizing homocysteine from its binding proteins (albumin). (drprincetta.com)
- Further, some patients may have genetic enzyme deficiencies that may impair homocysteine metabolism. (patientoneformulas.com)
- Because Quatrefolic is active when it enters the body, it is better able to influence pathways for homocysteine metabolism. (patientoneformulas.com)
- Trimethylglycine, also known as betaine, is considered a key player in homocysteine metabolism and has long been administered as a methyl donor for remethylating homocysteine back into methionine. (patientoneformulas.com)
- Trimethylglycine (TMG) betaine is a necessary cofactor in the secondary pathway of homocysteine metabolism. (patientoneformulas.com)
- In order to fully comprehend the link between folate and early prenatal development, it is necessary to examine the biochemical roles of folate in homocysteine metabolism. (ppdictionary.com)
Enzyme4
- CBS is a pivotal enzyme that is essential for the management of methionine and cysteine in the body. (travere.com)
- 2007). In the latter mechanism, homocysteine is condensed with the amino acid serine in a reaction catalyzed by the enzyme cystathionine-β-synthase to form cystathionine, which in turn, is reduced to cysteine and α-ketobutyrate by the enzyme cystathionine lyase. (ppdictionary.com)
- During the remethylation into methionine, a methyl group is transferred from 5-methyltetrahydrofolate to homocysteine via the enzyme methionine synthase. (ppdictionary.com)
- Electron cryoEM structures of mitochondrial, chloroplast, and bacterial ATP synthases have revealed the architecture of the FO region, helping to explain the mechanisms of proton translocation, dimerization of the enzyme in mitochondria, and cristae formation. (tcdb.org)
Serine3
- In transsulfuration pathway, Hcy is reacted with serine (Ser) to form cystathionine via vitamin B 6 -dependent cystathionine β -synthase (CBS). (hindawi.com)
- In the protein-protein interaction (PPI) network, most of the interacting proteins were serine acetyltransferases and cysteine synthases, which are involved in the cysteine synthesis pathway. (bvsalud.org)
- CONCLUSION: On the basis of the PPI network for the significantly enriched genes in the pathways which were specifically enriched at different time points in ZH, we hypothesize that serine acetyltransferases and cysteine synthases are crucial for the cysteine-related resistance of peanut to web blotch. (bvsalud.org)
Glutamine Synthase1
- Ammonia incorporation in animals occurs through the actions of glutamate dehydrogenase and glutamine synthase. (medmuv.com)
Oxidative5
- The researchers attributed the protection to H2S opening K-ATP channels, scavenging radical oxidative species (ROS), and possibly lowering metabolism. (gotomydoctor.com)
- Parasitic ecule because it reversibly binds to certain proteins, such as kinetoplastid flagellates represent a rare example of organisms transcription factors and ion channels, and thus modulates their that depend on oxidative metabolism but are heme auxotrophs. (medicinelakex1.com)
- Cysteine has a very high degree of reactivity, essentially undergoing numerous oxidative reactions (1). (metabolichealing.com)
- Ferroptosis acts as an independent pathway for suppressing tumor growth and is tightly connected with metabolism and oxidative stress responses. (nature.com)
- The corresponding genes for the DFS-SEs were significantly enriched in mitochondria and their associated pathways according to Gene Ontology annotation and in the pathways of fatty acid metabolism, oxidative phosphorylation and Huntington's disease according to a Kyoto Encyclopedia of Genes and Genomes pathway analysis. (spandidos-publications.com)
Pathways4
- Two other interrelated pathways of methionine metabolism can produce accumulation of homocysteine and its metabolites: defective methylcobalamin synthesis (homocystinuria II) and abnormality in MTHFR (homocystinuria III). (mhmedical.com)
- and ether lipid metabolism are specific to the active defense of ZH against YY187, whereas porphyrin metabolism as well as taurine and hypotaurine metabolism are pathways specifically involved in the passive defense of ZH against YY187. (bvsalud.org)
- The CcpA regulon comprises genes involved in sugar uptake, fermentation and amino acids metabolism, confirming the role of CcpA as a link between carbon and nitrogen pathways. (studyres.com)
- Because of this central role in cellular metabolism, a precise knowledge of the biosynthetic pathways that are responsible for homeostatic regulation of methionine and AdoMet in plants has practical implications, particularly in herbicide design. (rhumbarlv.com)
Proteins3
- metabolism of this heme-free eukaryote, we searched for heme- There are only a few examples of facultatively anaerobic or containing proteins in its de novo sequenced genome and exam-ined several cellular processes for which heme has so far been con- pathogenic bacteria that do not require heme (10-12), but no sidered indispensable. (medicinelakex1.com)
- Folates are involved in a large number of biochemical processes, such as amino acid metabolism, purine and pyrimidine synthesis, and methylation of a large number of nucleic acids, deoxyribonucleic acid, proteins, and lipids (Alberto et al . (ppdictionary.com)
- Homocysteine is a homologue of the amino acid cysteine which is used by the body to help manufacture proteins and carry out cellular metabolism. (ppdictionary.com)
Deficiency4
- However, chronic alcohol consumption decreases GSH generation through cysteine deficiency by suppressing the methionine cycle and trans-sulfuration system, whereas it turns on an alternative defense pathway, such as the xCT transporter, to compensate for GSH shortage. (e-cmh.org)
- Deficiency of cystathionine synthase (homocystinuria I) leads to a failure of transsulfuration of precursors of cysteine, an important component of collagen. (mhmedical.com)
- However, when normal metabolism is disturbed, due to deficiency of cystathionine-b-synthase, which requires vit B6 for activation, Hcy is accumulated in the blood with an increase of methionine, resulting into mental retardation (homocystinuria type I). A decrease of cysteine may cause eye diseases, due to decrease in the synthesis of glutathione (antioxidant). (niscpr.res.in)
- Others are more common and are treated with folate, vit B12 and in selected cases as in methionine synthase deficiency, methionine, avoiding excess. (niscpr.res.in)
Pathway3
- In remethylation pathway, Hcy can be remethylated to form Met via methionine synthase (MS) or betaine-homocysteine methyltransferase (BHMT), in which cofactors such as folic acid and vitamin B 12 or betaine are required. (hindawi.com)
- B-12 also works in concert with the coenzyme methionine synthase, creating a primary pathway of remethylation of homocysteine. (patientoneformulas.com)
- As an example of one of the complex transformations on this pathway, the figure below shows the structure of the pyrimidine synthase catalyzing the complex rearrangement of aminoimidazole ribotide (left) to the thiamin pyrimidine (right). (tamu.edu)
Nitric5
- Among various mechanisms, the uncoupling of nitric oxide synthase (NOS) in vascular cells has also widely been reported to be involved in ROS generation. (hindawi.com)
- Intracellular calcium determines the activation of endothelial nitric oxide synthase (eNOS), with the production of higher quantities of nitric oxide. (allmaxnutrition.com)
- Nitric oxide synthase enzymes catalyze the formation of nitric oxide from L-arginine through an NADPH- and oxygen-dependent mechanism. (thermofisher.com)
- Vasorelaxation, neurotransmission and cytotoxicity can all be potentiated through cellular response to NO. NO production is mediated by members of the nitric oxide synthase (NOS) family. (thermofisher.com)
- oxLDL can interfere with the transduction of the signals responsible for triggering the activation of endothelial nitric oxide synthase (eNOS), causing reduced vasodilation and endothelial dysfunction, which are the main characteristics of preeclampsia. (biomedcentral.com)
Protein5
- d) It is highly significant in the liver and kid-neys of birds which excrete uric acid as the end product of purine metabolism and also of protein and amino acid catabolism. (biologydiscussion.com)
- Further fine mapping in a large population with over 1,000 F 2 plants narrowed this region to 39.08 Kb harboring two genes, Cla97C01G008760 and Cla97C01G008770 , which encode phytoene synthase ( ClPsy1 ) and GATA zinc finger domain-containing protein, respectively. (researchsquare.com)
- A gene controlling the golden flesh trait in watermelon was firstly discovered and fine-mapped to a 39.08-Kb region on chromosome 1 through a forward genetic strategy, and Cla97C01G008760 (annotated as phytoene synthase protein, ClPsy1 ) was recognized as the most likely candidate gene. (researchsquare.com)
- Ammonia the highly toxic product of protein catabolism, is rapidly inactivated by a variety of reactions. (medmuv.com)
- and ii) use of glycogen synthase kinase 3β inhibitors to maintain high protein and activity levels of Nrf2 in the nucleus. (drperlmutter.com)
Trans-sulfuration1
- When homocysteine accumulates in cells, it is removed by remethylation into the amino acid methionine or by a process known as trans-sulfuration into cysteine (Alberto et al . (ppdictionary.com)
Lipid metabolism1
- In PE, lipid metabolism is altered. (biomedcentral.com)
Uric acid1
- A frequently overlooked "waste product" of metabolism, uric acid actually threatens our health. (drperlmutter.com)
Methanethiol2
- Methanethiol (CH3 SH) - Is an organosulfur compound derived from cysteine oxidation. (metabolichealing.com)
- The main backbone is derived from aspartic acid, while the sulfur may come from cysteine, methanethiol, or hydrogen sulfide. (rhumbarlv.com)
Derived from methionine1
- Homocysteine (Hcy) is a nonprotein amino acid, derived from methionine (Met) metabolism [ 1 ]. (hindawi.com)
Mitochondria2
- During general alcohol metabolism, hepatocytes generate mitochondria- and cytochrome P450 2E1 (CYP2E1)-mediated reactive oxygen species (ROS) whose accumulation elicits activation of the hepatic anti-oxidant system, including glutathione (GSH). (e-cmh.org)
- compared the structural and functional properties of F-ATP synthases in plant mitochondria with those of yeast and mammals. (tcdb.org)
Genetic4
- Hyperhomocysteinemia (HHcy) is associated with genetic and nutritional abnormalities in Hcy metabolism and has been demonstrated to be an independent risk factor for cardiovascular disease and several other diseases [ 4 - 7 ]. (hindawi.com)
- Understanding your genetic mutations (SNPs) will help you identify which processes and enzymes may need support in your body and your metabolism. (doctordoni.com)
- The majority of KEGG-annotated DEGs were involved in metabolism, genetic information processing, cellular processes, the organismal system, human diseases and environmental information processing. (biomedcentral.com)
- Biochemical genetic testing and newborn screening are essential laboratory services for the screening, detection, diagnosis, and monitoring of inborn errors of metabolism or inherited metabolic disorders. (cdc.gov)
Acceptor1
- a) Enzymes that catalyse the removal of hy-drogen from a substrate but use only oxy-gen as a hydrogen acceptor to form water as a reaction product (with the exception of uricase and monoamine oxidase which form H 2 O 2 ). (biologydiscussion.com)
Reaction2
- In plants, this reaction is catalyzed by a cobalamin-independent methionine synthase (EC 2.1. (rhumbarlv.com)
- Thus, methionine synthase not only catalyzes the last reaction in de novo methionine synthesis but also serves to regenerate the methyl group of AdoMet. (rhumbarlv.com)
Hepatic2
- The mRNA expression and enzymatic activity of cystathionine β -synthase and cystathionine γ -lyase were upregulated after quercetin, methionine, or quercetin plus methionine treatment and a more significant increase was observed for hepatic cystathionine β -synthase in the methionine plus quercetin treated rats, suggesting an interaction between methionine and quercetin. (hindawi.com)
- In addition, we address different responses of alcohol metabolism that are specific to hepatic zones ( Fig. 1 ). (e-cmh.org)
Sulfur4
- What is being dealt with is sulfur metabolism and utilization. (metabolichealing.com)
- Cysteine is the sulfur-containing amino acid that performs some critical functions in the body, including the formation of thiol-bearing antioxidants such as glutathione and metallothionein. (metabolichealing.com)
- As a result of sulfur metabolism, a number of intermediate metabolites are produced downstream of CBS. (metabolichealing.com)
- Some bacteria can synthesize methionine using organic sulfur through transsulfuration of O-succinyl-L-homoserine with L-cysteine to form L-cystathionine. (rhumbarlv.com)
Vitamin1
- Subsequently, cystathionine is hydrolyzed to yield cysteine (Cys) and α -ketobutyrate via vitamin B 6 -dependent cystathionine γ -lyase (CSE) and finally to taurine (Tau) [ 2 , 3 ]. (hindawi.com)
Folate2
- Patients with MTHFR have folate needs that vary quite a bit from patient to patient, so there will not be a one perfect product for them. (drprincetta.com)
- In normal circumstances, it is converted to cysteine and partly remethylated to methionine with the help of vit B12 and folate. (niscpr.res.in)
Biochemical processes1
- Reactive oxygen species (ROS) are produced as a result of aerobic metabolism and as by-products through numerous physiological and biochemical processes. (hindawi.com)
Typically1
- Although historically the term often referred to a physical trait or physiological metric, it now typically refers to products of genomic, metabolomic, and proteomic research. (medscape.com)
Lipids1
- Existing studies have confirmed that ferroptosis involves the metabolisms of the Fe, lipids, amino acid, each mechanism is mutually independent but interrelated, and they are formed a complex regulatory network. (frontiersin.org)
Mechanism1
- pH-dependent 11 degrees F1FO ATP synthase sub-steps reveal aspects of the FO torque generating mechanism ( Yanagisawa and Frasch 2021 ). (tcdb.org)
Glucose1
- These include reactions of cellular respiration, the release of glucose stores, and amino acid metabolism. (mskcc.org)
Metabolic1
- A combination of Citrulline Malate (2:1), Agmatine Sulfate, GLYCERPUMP, N-Acetyl-L-Cysteine (NAC) and Taurine have been included at high levels to take full advantage of their ability to increase the body's physiological tendency to flood muscles with blood, nutrients, oxygen and yes, metabolic waste products in response to intense training. (allmaxnutrition.com)
Lysine1
- Lysine plays a significant role in growth and metabolism. (targetwoman.com)
Secondary2
- This tRNA is unnecessary for growth, but is required for some aspects of secondary metabolism and morphological development. (biomedcentral.com)
- Our results revealed unexpected effects of bldA mutation on gene expression during growth and the transition phase that precedes entry into stationary phase, as well as more wide-ranging effects on secondary metabolism than previously suspected, and led to further exploration of some of the underpinning mechanisms. (biomedcentral.com)
Role3
- Thiamin pyrophosphate plays a key role in the stabilization of the acyl carbanion synthon in carbohydrate and amino acid metabolism. (tamu.edu)
- N-acetyl cysteine is an antioxidant that might play a role in preventing cancer. (medlineplus.gov)
- As L carnitine is similar to another amino acid Carnitine which plays a major role in energy metabolism. (targetwoman.com)
Bone2
- Bone is an active metabolism tissue, which constantly undergoes bone remodeling. (frontiersin.org)
- Bone resorption occurs mainly on the bone surface, by the osteoclasts (OC) on bone metabolism in continuous absorption. (frontiersin.org)
Roles1
- and some have probable roles in reorganising metabolism after rapid growth. (biomedcentral.com)
Form3
- Inhaling a prescription form of N-acetyl cysteine helps treat collapsed lungs caused by mucus blockage. (medlineplus.gov)
- Inhaling a prescription form of N-acetyl cysteine is helpful to prepare people for diagnostic lung tests. (medlineplus.gov)
- Inhaling a prescription form of N-acetyl cysteine helps prevent crusting in people with a tube in the windpipe. (medlineplus.gov)
Kidney1
- Taking N-acetyl cysteine by mouth, with or without other drugs, might help to prevent kidney problems caused by dyes used during some X-ray exams. (medlineplus.gov)
Determines1
- In the 1980s, a foetal programming concept emerged that suggested that the intrauterine environment determines and programmes physiology and metabolism throughout life [ 8 ]. (biomedcentral.com)
Body1
- N-Acetyl-L-Cysteine (NAC) increases the amount of erythropoietin (EPO) in the body, in turn increasing red blood cell production. (allmaxnutrition.com)