Read by QxMD icon Read

L gulonolactone oxidase enzyme

Lucie Aumailley, Marie Julie Dubois, Tracy A Brennan, Chantal Garand, Eric R Paquet, Robert J Pignolo, André Marette, Michel Lebel
Werner syndrome (WS) is a premature aging disorder caused by mutations in a RecQ-family DNA helicase (WRN). Mice lacking part of the helicase domain of the WRN ortholog exhibit several phenotypic features of WS. In this study, we generated a Wrn mutant line that, like humans, relies entirely on dietary sources of vitamin C (ascorbate) to survive, by crossing them to mice that lack the gulonolactone oxidase enzyme required for ascorbate synthesis. In the presence of 0.01% ascorbate (w/v) in drinking water, double-mutant mice exhibited a severe reduction in lifespan, small size, sterility, osteopenia, and metabolic profiles different from wild-type (WT) mice...
February 8, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
Biyun Ching, Shit F Chew, Yuen K Ip
Biosynthesis of ascorbate is known to occur in liver and/or kidney of some vertebrates; however, a recent study discovered the expression of l-gulono-γ-lactone oxidase, an enzyme essential for ascorbate synthesis, in the brain of the African lungfish, Protopterus annectens. This report provides an up-to-date review on ascorbate synthesis in fishes and the possible future directions of study in view of the discovery of the unusual site of ascorbate biosynthesis.
February 2015: IUBMB Life
Glen Wheeler, Takahiro Ishikawa, Varissa Pornsaksit, Nicholas Smirnoff
Ascorbic acid (vitamin C) is an enzyme co-factor in eukaryotes that also plays a critical role in protecting photosynthetic eukaryotes against damaging reactive oxygen species derived from the chloroplast. Many animal lineages, including primates, have become ascorbate auxotrophs due to the loss of the terminal enzyme in their biosynthetic pathway, L-gulonolactone oxidase (GULO). The alternative pathways found in land plants and Euglena use a different terminal enzyme, L-galactonolactone dehydrogenase (GLDH)...
2015: ELife
Yue Huang, Xiaodong Zhu, Lishi Wang, Xiaoyun Liu, Lu Lu, Weikuan Gu, Yan Jiao
The objective of this study is to identify sex differentially expressed genes in bone using a mouse model of spontaneous fracture, sfx, which lacks the gene for L-gulonolactone oxidase (Gulo), a key enzyme in the ascorbic acid (AA) synthesis pathway. We first identified the genes that are differentially expressed in the femur between female and male in sfx mice. We then analyzed the potential gene network among those differentially expressed genes with whole genome expression profiles generated using spleens of female and male mice of a total of 67 BXD (C57BL/6J X DBA/2J) recombinant inbred (RI) and other strains...
2014: TheScientificWorldJournal
Biyun Ching, Jasmine L Y Ong, You Rong Chng, Xiu Ling Chen, Wai P Wong, Shit F Chew, Yuen K Ip
This study aimed to test the hypothesis that the brain of Protopterus annectens expressed L-gulono-γ-lactone oxidase (gulo/Gulo), the enzyme catalyzing the last step of ascorbate biosynthesis, and could maintain high concentrations of ascorbate during estivation. We cloned and sequenced gulo from the kidney of P. annectens and performed quantitative PCR to determine its mRNA expression in kidney and brain. Gulo activity was assayed and its protein abundance was determined by Western blot using custom-made anti-Gulo antibody...
August 2014: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
Yayoi Kagami, Yoshitaka Kondo, Setsuko Handa, Naoki Maruyama, Akihito Ishigami
Senescence marker protein-30 (SMP30) was first described as a physiologic entity that decreases in the rat liver and kidney with aging. Previously, we established that SMP30 is the lactone-hydrolyzing enzyme gluconolactonase (GNL), which is involved in ascorbic acid (AA) biosynthesis. In the present study, we found SMP30/GNL mRNA expressed in the mouse ovary. To ascertain the reason for ovarian SMP30/GNL expression, we examined mice during gestation. SMP30/GNL mRNA expression was evident at the start of gestation, increased for the next eight days then decreased rapidly...
2013: Biological & Pharmaceutical Bulletin
Bernard J Fisher, Donatas Kraskauskas, Erika J Martin, Daniela Farkas, Puneet Puri, H Davis Massey, Michael O Idowu, Donald F Brophy, Norbert F Voelkel, Alpha A Fowler, Ramesh Natarajan
BACKGROUND: Multiple organ dysfunction syndrome (MODS) is the principal cause of death in patients with sepsis. Recent work supports the notion that parenteral vitamin C (VitC) is protective in sepsis through pleiotropic mechanisms. Whether suboptimal levels of circulating VitC increase susceptibility to sepsis-induced MODS is unknown. MATERIALS AND METHODS: Unlike mice, humans lack the ability to synthesize VitC because of loss of L-gulono-γ-lactone oxidase (Gulo), the final enzyme in the biosynthesis of VitC...
September 2014: JPEN. Journal of Parenteral and Enteral Nutrition
Seyeon Bae, Chung-Hyun Cho, Hyemin Kim, Yejin Kim, Hang-Rae Kim, Young-Il Hwang, Jung Hwan Yoon, Jae Seung Kang, Wang Jae Lee
AIM: l-ascorbic acid (vitamin C) insufficiency is considered one of the major risk factors for the development of liver disease. However, its specific effects and related mechanisms in vivo are largely unknown. The objective of this study was to investigate the in vivo protective role of vitamin C and its related mechanisms in liver injury with Gulo(-/-) mice that cannot synthesize vitamin C like humans due to the lack of l-gulonolactone-γ-oxidase (Gulo), an essential enzyme for vitamin C synthesis...
December 10, 2013: Antioxidants & Redox Signaling
Y Chen, C P Curran, D W Nebert, K V Patel, M T Williams, C V Vorhees
Organisms using oxygen for aerobic respiration require antioxidants to balance the production of reactive oxygen species during metabolic processes. Various species--including humans and other primates--suffer mutations in the GULO gene encoding L-gulono-γ-lactone oxidase; GULO is the rate-limiting enzyme in the biosynthesis of ascorbate, an important cellular antioxidant. Animals lacking the ability to synthesize vitamin C develop scurvy without dietary supplementation. The Gulo-/- knockout (KO) mouse requires oral supplemental vitamin C; without this supplementation the animal dies with a scorbutic condition within several weeks...
April 2012: Genes, Brain, and Behavior
Jie Cui, Xinpu Yuan, Lina Wang, Gareth Jones, Shuyi Zhang
The traditional assumption that bats cannot synthesize vitamin C (Vc) has been challenged recently. We have previously shown that two Old World bat species (Rousettus leschenaultii and Hipposideros armiger) have functional L-gulonolactone oxidase (GULO), an enzyme that catalyzes the last step of Vc biosynthesis de novo. Given the uncertainties surrounding when and how bats lost GULO function, exploration of gene evolutionary patterns is needed. We therefore sequenced GULO genes from 16 bat species in 5 families, aiming to establish their evolutionary histories...
2011: PloS One
Neha Biyani, Rentala Madhubala
Plants and most animals can synthesize ascorbate (vitamin C) for their own requirements, but humans have lost this ability during evolution. The last step in the biosynthesis of L-ascorbic acid involves the conversion of an aldonolactone substrate to ascorbate (or analogues), reactions catalyzed by a family of flavoprotein aldonolactone oxidase/dehydrogenases. We report cloning, molecular characterization, localization and functional importance of arabinonolactone oxidase (LdALO), an enzyme from L. donovani, a protozoan parasite that causes visceral leishmaniasis...
December 2011: Molecular and Biochemical Parasitology
Katarzyna J Nytko, Nobuyo Maeda, Philipp Schläfli, Patrick Spielmann, Roland H Wenger, Daniel P Stiehl
Prolyl-4-hydroxylation is necessary for proper structural assembly of collagens and oxygen-dependent protein stability of hypoxia-inducible transcription factors (HIFs). In vitro function of HIF prolyl-4-hydroxylase domain (PHD) enzymes requires oxygen and 2-oxoglutarate as cosubstrates with iron(II) and vitamin C serving as cofactors. Although vitamin C deficiency is known to cause the collagen-disassembly disease scurvy, it is unclear whether cellular oxygen sensing is similarly affected. Here, we report that vitamin C-deprived Gulo(-/-) knockout mice show normal HIF-dependent gene expression...
May 19, 2011: Blood
Arash Akbarzadeh, Hamid Farahmand, Frouzandeh Mahjoubi, Mohammad Ali Nematollahi, Piia Leskinen, Kalle Rytkönen, Mikko Nikinmaa
l-Gulono-gamma-lactone oxidase (GULO) is a key enzyme for the biosynthesis of ascorbate, which is essential for several cellular functions. In the present study, mRNA expression of GULO gene was evaluated during the early development of Persian sturgeon. First, because there are no comparative studies that have established suitable quantitative real-time PCR reference genes in sturgeons for any physiological conditions, we evaluated six candidate reference genes (ACTB, RPL13, UBQ, RPL6, GAPDH and EF1A) during the early development of Persian sturgeon...
April 2011: Comparative Biochemistry and Physiology. Part B, Biochemistry & Molecular Biology
Marc Y Lachapelle, Guy Drouin
The capacity to biosynthesize ascorbic acid has been lost in a number of species including primates, guinea pigs, teleost fishes, bats, and birds. This inability results from mutations in the GLO gene coding for L-gulono-γ-lactone oxidase, the enzyme responsible for catalyzing the last step in the vitamin C biosynthetic pathway. We analyzed available primate and rodent GLO gene sequences to determine their evolutionary history. We used a method based on sequence comparisons of lineages with and without functional GLO genes to calculate inactivation dates of 61 and 14 MYA for the primate and guinea pig genes, respectively...
February 2011: Genetica
Nuria Martí, Pedro Mena, Jose Antonio Cánovas, Vicente Micol, Domingo Saura
The literature on the content and stability of vitamin C (ascorbic acid, AA) in citrus juices in relation to industrial practices is reviewed. The role of vitamin C from citrus juices in human diet is also reviewed. Citrus fruits and juices are rich in several types of bioactive compounds. Their antioxidant activity and related benefits derive not only from vitamin C but also from other phytochemicals, mainly flavonoids. During juice processing, temperature and oxygen are the main factors responsible for vitamin C losses...
May 2009: Natural Product Communications
Chung-Mu Park, Chung-Won Cho, Michael E Rosenfeld, Young-Sun Song
The present study was designed to investigate whether hyperhomocysteinemia (HHcy) induced by methionine supplementation promotes oxidative stress and nuclear factor kappaB (NFkappaB) activation in livers of C57BL/6 mice when fed a 2% methionine and low folate (1 mg/kg) diet for 12 weeks. Plasma homocysteine concentrations of mice fed methionine were found to be 49 micromol/L by 12 weeks of feeding, which was five times higher than that of controls. HHcy induced by methionine feeding significantly increased oxidative stress, as measured by thiobarbituric acid-reactive substances (P < ...
December 2008: Journal of Medicinal Food
Jian Yan, Yan Jiao, Xinmin Li, Feng Jiao, Wesley G Beamer, Cliff J Rosen, Weikuan Gu
Humans and guinea pigs are species which are unable to synthesize ascorbic acid (vitamin C) because, unlike rodents, they lack the enzyme L-gulonolactone oxidase (Gulo). Although the phenotype of lacking vitamin C in humans, named scurvy, has long been well known, information on the impact of lacking Gulo on the gene expression profiles of different tissues is still missing. This knowledge could improve our understanding of molecular pathways in which Gulo may be involved. Recently, we discovered a deletion that includes all 12 exons in the gene for Gulo in the sfx mouse, characterized by spontaneous bone fractures...
2007: Genetics and Molecular Biology
Flora J Logan, Martin C Taylor, Shane R Wilkinson, Harparkash Kaur, John M Kelly
Humans lack the ability to synthesize vitamin C (ascorbate) due to the absence of gulonolactone oxidase, the last enzyme in the biosynthetic pathway in most other mammals. The corresponding oxidoreductase in trypanosomes therefore represents a target that may be therapeutically exploitable. This is reinforced by our observation that Trypanosoma cruzi, the causative agent of Chagas' disease, lacks the capacity to scavenge ascorbate from its environment and is therefore dependent on biosynthesis to maintain intracellular levels of this vitamin...
November 1, 2007: Biochemical Journal
Sucheta Telang, Amy L Clem, John W Eaton, Jason Chesney
Angiogenesis requires the deposition of type IV collagen by endothelial cells into the basement membrane of new blood vessels. Stabilization of type IV collagen triple helix depends on the hydroxylation of proline, which is catalyzed by the iron-containing enzyme prolyl hydroxylase. This enzyme, in turn, requires ascorbic acid to maintain the enzyme-bound iron in its reduced state. We hypothesized that dietary ascorbic acid might be required for tumor angiogenesis and, therefore, tumor growth. Here, we show that, not surprisingly, ascorbic acid is necessary for the synthesis of collagen type IV by human endothelial cells and for their effective migration and tube formation on a basement membrane matrix...
January 2007: Neoplasia: An International Journal for Oncology Research
Young Sun Cho, Susan E Douglas, Jeffrey W Gallant, Keun Yong Kim, Dong Soo Kim, Yoon Kwon Nam
Most advanced teleosts lack L-gulono-gamma-lactone oxidase (GULO), a key enzyme required for the biosynthesis of ascorbic acid. However, extant representatives of primitive species including sturgeon and many cartilaginous fishes, are exceptional in their ability to synthesize ascorbic acid de novo. In the present study, full-length GULO cDNAs were isolated from white sturgeon (Acipenser transmontanus) and two shark species belonging to the Triakidae (Triakis scyllium and Mustelus manazo). The open reading frames from all three species contained 440 amino acids and the deduced polypeptides had similar hydropathy profiles, predicted molecular masses and theoretical pI values...
June 2007: Comparative Biochemistry and Physiology. Part B, Biochemistry & Molecular Biology
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"