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Cyclic N-OxidesSpin TrappingPyrroline Carboxylate ReductasesSpin LabelsFree RadicalsElectron Spin Resonance Spectroscopy1-Pyrroline-5-Carboxylate DehydrogenaseNitrogen OxidesHydroxyl RadicalCarboxylic AcidsPeroxidesPyrrolesHydroxidestert-ButylhydroperoxideOxidation-ReductionFree Radical ScavengersHydrogen PeroxidePentose Phosphate PathwayMolecular StructureSuperoxidesHorseradish PeroxidaseCarbon-Carbon LyasesKineticsPyrrolidonecarboxylic AcidOxygenProline OxidaseModels, ChemicalProlineLyasesSuperoxide DismutaseOseltamivirPyroglutamate HydrolaseOrnithine-Oxo-Acid TransaminaseOxidoreductases Acting on CH-NH Group DonorsGlutamate-5-Semialdehyde DehydrogenaseModels, MolecularCarbolinesCrystallography, X-RayBinding SitesPipecolic AcidsCatalysisThiazolidinesAmino Acids, CyclicStereoisomerismortho-AminobenzoatesAmino Acid SequenceCrotonatesSubstrate SpecificityMolecular Sequence DataEthylenesL-Lysine 6-TransaminaseStructure-Activity RelationshipMagnetic Resonance SpectroscopyMethanospirillumHydrogen-Ion ConcentrationProtein ConformationDeltaproteobacteriaAspartic AcidRhodiumCyclohexanecarboxylic AcidsEstersCatalytic DomainHydrogen BondingMutagenesis, Site-DirectedMolecular ConformationDecarboxylationProtonsgamma-GlutamylcyclotransferaseLigands2-Aminoadipic AcidAlkenesZanamivirProteaceaeBenzoatesOrnithineEscherichia coliGlutamic AcidAlkanesulfonatesAnionsPyransGlutamatesPhosphotransferases (Carboxyl Group Acceptor)Protein BindingFlumazenilLysineHalogensStatic ElectricityNonheme Iron ProteinsPlant ExudatesHistidinePicolinic AcidsArginineAcetalsProtein Structure, SecondaryChloridesWaterCyclizationCyclopropanesSpectroscopy, Fourier Transform InfraredOrganosilicon Compounds
DehydrogenaseReductase familyALDH4A1MitochondrialPYCR1NADPHDeficiencyEnzyme that catalyzesCatalyzesHyperprolinemiaChromosomeGeneMitochondriaReactionSequenceGlutamate metabolismPYCR2HGNCOxidoreductaseConvertsGamma-semialdehydeCompoundsArabidopsisPhosphatePathwayCysteine dioxygenaseHumanBiosynthesisBacteriaProteinsDependentFAMILYSymptomsNovoVitro
Dehydrogenase16
  • In enzymology, a 1-pyrroline-5-carboxylate dehydrogenase (EC 1.2.1.88) is an enzyme that catalyzes the chemical reaction (S)-1-pyrroline-5-carboxylate + NAD+ + 2 H2O ⇌ {\displaystyle \rightleftharpoons } L-glutamate + NADH + H+ The three substrates of this enzyme are (S)-1-pyrroline-5-carboxylate, NAD+, and H2O, whereas its three products are glutamate, NADH, and H+. (wikipedia.org)
  • Other names in common use include delta-1-pyrroline-5-carboxylate dehydrogenase, 1-pyrroline dehydrogenase, pyrroline-5-carboxylate dehydrogenase, pyrroline-5-carboxylic acid dehydrogenase, L-pyrroline-5-carboxylate-NAD+ oxidoreductase, and 1-pyrroline-5-carboxylate:NAD+ oxidoreductase. (wikipedia.org)
  • The ALDH4A1 gene provides instructions for producing the enzyme pyrroline-5-carboxylate dehydrogenase, which is found in tissues throughout the body. (medlineplus.gov)
  • Pyrroline-5-carboxylate dehydrogenase starts the second step in the process that breaks down the protein building block (amino acid) proline. (medlineplus.gov)
  • ALDH4A1 gene variants reduce or eliminate the function of the pyrroline-5-carboxylate dehydrogenase enzyme. (medlineplus.gov)
  • A lack of pyrroline-5-carboxylate dehydrogenase function leads to decreased breakdown of proline and elevated levels of proline and intermediate breakdown product pyrroline-5-carboxylate, causing the signs and symptoms of hyperprolinemia type II. (medlineplus.gov)
  • Hu CA, Lin WW, Valle D. Cloning, characterization, and expression of cDNAs encoding human delta 1-pyrroline-5-carboxylate dehydrogenase. (medlineplus.gov)
  • Crystal structure of Thermus thermophilus Delta1-pyrroline-5-carboxylate dehydrogenase. (medlineplus.gov)
  • In particular, it is synthesized via the oxidation of proline by pyrroline-5-carboxylate reductase 1 (PYCR1) (EC 1.5.1.2) or by proline dehydrogenase (PRODH) (EC 1.5.99.8). (hmdb.ca)
  • It is hydrolyzed into L-glutamate by delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) (EC 1.5.1.12). (hmdb.ca)
  • Notably, mice infected with fungal cells unable to catabolize proline, e.g., cells lacking the Put2 enzyme (1-pyrroline-5-carboxylate (P5C) dehydrogenase) , showed milder illness or no symptoms at all. (sciencedaily.com)
  • alcohol dehydrogenase 5 (class III), c. (gsea-msigdb.org)
  • dehydrogenase/reductase 1 [Source:HGNC. (gsea-msigdb.org)
  • Hyperprolinemia is a condition which occurs when the amino acid proline is not broken down properly by the enzymes proline oxidase or pyrroline-5-carboxylate dehydrogenase , causing a buildup of proline in the body. (mdwiki.org)
  • Hyperprolinemia type II is caused by a mutation in the ALDH4A1 gene, for the enzyme 1-pyrroline-5-carboxylate dehydrogenase . (mdwiki.org)
  • A deficiency of either proline oxidase or pyrroline-5-carboxylate dehydrogenase results in a buildup of proline in the body. (mdwiki.org)
Reductase family2
  • This gene belongs to the pyrroline-5-carboxylate reductase family. (nih.gov)
  • aldo-keto reductase family 1 member C2. (gsea-msigdb.org)
ALDH4A11
Mitochondrial1
  • The encoded mitochondrial protein catalyzes the conversion of pyrroline-5-carboxylate to proline, which is the last step in proline biosynthesis. (nih.gov)
PYCR12
  • Pyrroline-5-carboxylate reductase 1 (PYCR1) plays an important role in tumor development. (jcancer.org)
  • RNA interference was used to inhibit the expression of PYCR1 in PANC- 1 and AsPC-1 cells. (jcancer.org)
NADPH1
  • PYCR catalyzes an NADPH dependent reaction of pyrroline-5-carboxylate (P5C) into proline. (jcancer.org)
Deficiency3
  • Pyrroline-5-carboxylate reductase 2 (PYCR2) deficiency causes hereditary spastic paraplaegia in late childhood. (nih.gov)
  • Chronically high levels of pyrroline-5-carboxylate are associated with at least five inborn errors of metabolism, including hyperprolinemia type I, hyperprolinemia type II, iminoglycinuria, prolinemia type II, and pyruvate carboxylase deficiency. (hmdb.ca)
  • A deficiency of the latter enzyme leads to higher levels of proline and a buildup of the intermediate breakdown product pyrroline-5-carboxylate, causing the signs and symptoms of hyperprolinemia type II.Hyperprolinemia is inherited in an autosomal recessive pattern, which means two copies of the gene in each cell are altered. (mdwiki.org)
Enzyme that catalyzes2
  • Pyrroline-5-carboxylate reductase (PYCR) is a proline biosynthetic enzyme that catalyzes the NAD(P)H-dependent reduction of Δ-pyrroline-5-carboxylate (P5C) to proline. (anl.gov)
  • An enzyme that catalyzes the oxidation of 1-pyrroline-5-carboxylate to L-GLUTAMATE in the presence of NAD. (bvsalud.org)
Catalyzes3
  • The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. (anticorps-enligne.fr)
  • Catalyzes the reduction of 1-pyrroline-5-carboxylate (PCA) to L-proline. (expasy.org)
  • Delta 1-pyrroline-5-carboxylate reductase, catalyzes the final step in proline biosynthesis from glutamate and ornithine.In situ hybridization indicated that under normal growth conditions, the highest concentration of P5CR transcripts occurs in the cortical parenchyma, phloem, vascular cambium and pith parenchyma in the vicinity of the protoxylem. (gifu-u.ac.jp)
Hyperprolinemia2
  • Hyperprolinemia type II results in high levels of pyrroline-5-carboxylate. (hmdb.ca)
  • Hyperprolinemia type II results in proline levels in the blood between 10 and 15 times higher than normal, and high levels of a related compound called pyrroline-5-carboxylate . (mdwiki.org)
Chromosome1
Gene1
  • A soybean gene encoding delta 1-pyrroline-5-carboxylate reductase was isolated by functional complementation in Escherichia coli and is found to be osmoregulated. (expasy.org)
Mitochondria1
  • Pyrroline-5-carboxylate reductase (PYCR) is mainly located in the mitochondria and plays an important role in the biosynthesis of proline [ 10 , 11 ]. (jcancer.org)
Reaction2
  • This enzyme begins the process of degrading proline by starting the reaction that converts it to pyrroline-5-carboxylate. (mdwiki.org)
  • This enzyme helps to break down the pyrroline-5-carboxylate produced in the previous reaction, converting it to the amino acid glutamate . (mdwiki.org)
Sequence1
Glutamate metabolism1
  • The stereoisomer (S)-1-pyrroline-5-carboxylate is an intermediate in glutamate metabolism, arginine degradation, and proline biosynthesis and degradation. (hmdb.ca)
PYCR21
  • Disease variants of human Δ(1)-pyrroline-5-carboxylate reductase 2 (PYCR2). (nih.gov)
HGNC11
Oxidoreductase1
  • The systematic name of this enzyme class is (S)-1-pyrroline-5-carboxylate:NAD+ oxidoreductase. (wikipedia.org)
Converts1
  • This step converts pyrroline-5-carboxylate, which is produced in the first step, to the amino acid glutamate. (medlineplus.gov)
Gamma-semialdehyde2
  • 1-Pyrroline-5-carboxylic acid (CAS: 2906-39-0) is an enamine or an imino acid that forms upon the spontaneous dehydration of L-glutamate gamma-semialdehyde in aqueous solutions. (hmdb.ca)
  • S)-1-pyrroline-5-carboxylate, the product of the first enzyme of the pathway (EC 1.5.5.2 ) is in spontaneous equilibrium with its tautomer L-glutamate gamma-semialdehyde. (expasy.org)
Compounds1
Arabidopsis1
  • TRR14 is a novel protein important in trehalose (α-D-glucosyl-[1,1]-α-D-glucopyranoside) signaling in Arabidopsis. (oaji.net)
Phosphate2
  • In the budding yeast Saccharomyces cerevisiae , the synthesis of proline from glutamate in the cytoplasm is catalyzed by three enzymes: Pro1p (γ-glutamyl kinase), Pro2p (γ-glutamyl phosphate reductase), and Pro3p (Δ 1 -pyrroline-5-carboxylate (P5C) reductase) [6] [7] . (microbialcell.com)
  • Pyridoxal phosphate de-activation by pyrroline-5-carboxylic acid. (mdwiki.org)
Pathway2
  • Pyrroline-5-Carboxylate Reductase-2 Promotes Colorectal Cancer Progression via Activating PI3K/AKT/mTOR Pathway. (nih.gov)
  • Also, insulin pathway by producing phosphatidylinositol-3, 4, 5-triphosphate (PIP3), and EGF pathway by activation of GAB1, control the downstream events and lead to potentialities in the mitogenic signal. (oaji.net)
Cysteine dioxygenase1
Human3
  • Functional Impact of a Cancer-Related Variant in Human Δ-Pyrroline-5-Carboxylate Reductase 1. (anl.gov)
  • 2017 ) Resolving the Cofactor Binding Site in the Proline Biosynthetic Enzyme Human Pyrroline-5-Carboxylate Reductase 1. (academictree.org)
  • 2017 ) Correcting the record - cofactor binding of human pyrroline-5-carboxylate reductase Acta Crystallographica Section a Foundations and Advances . (academictree.org)
Biosynthesis1
  • [1] the ionic form is known as glutamate ) is an α- amino acid that is used by almost all living beings in the biosynthesis of protein s. (explained.today)
Bacteria1
  • Due to fossil resource shortage, butanol has been increasingly produced generally by a conventional process using anaerobic bacteria of the genus Clostridium [ 1 ]. (biomedcentral.com)
Proteins1
  • Microorganisms and plants are exposed to various environmental stresses during their lifespan and have developed a variety of adaptation strategies against these stresses (e.g., altered membrane composition, induction of stress-responsive proteins, and accumulation of compatible solutes) [1] . (microbialcell.com)
Dependent1
  • Thus, ABA-dependent and ABA-independent regulatory systems would buy TPCA-1 seem to exist, differentially controlling development, water content, and nitrogen metabolism under water deficit. (srcsignaling.com)
FAMILY2
Symptoms1
  • Pancreatic cancer is occult without specific symptoms, which means that many patients exhibit locally advanced disease or metastasis at the time of diagnosis [ 1 , 2 ]. (jcancer.org)
Novo1
  • In response to the elevated glutamate in the liver, the expression of metabotropic glutamate receptor 5 (mGluR5) is up-regulated in hepatic stellate cells (HSCs) along with enhanced production of 2-arachidonoylglycerol, which in turn stimulates cannabinoid receptor 1 (CB 1 R) on neighboring hepatocytes to increase de novo lipogenesis. (e-cmh.org)
Vitro1
  • These tissues were co-cultured with brain slices after the brain slices had first been maintained in vitro for 1 day, 5 days and 9 days. (oaji.net)