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Glutaric Acid

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https://www.readbyqxmd.com/read/30340205/hygroscopic-behavior-and-fractional-crystallization-of-mixed-nh-4-2-so-4-glutaric-acid-aerosols-by-vacuum-ftir
#1
Feng-Min Wu, Na Wang, Shu-Feng Pang, Yun-Hong Zhang
The hygroscopicity and phase transition of the mixed aerosol particles are significantly dependent upon relative humidity (RH) and interactions between particle components. Although the efflorescence behavior of particles has been studied widely, the crystallization behavior of each component in the particles is still poorly understood. Here, we study the hygroscopicity and crystallization behaviors of internally mixed ammonium sulfate (AS)/glutaric acid (GA) aerosols by a vacuum FTIR spectrometer coupled with a RH-controlling system...
October 11, 2018: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
https://www.readbyqxmd.com/read/30320856/complexation-of-pu-vi-with-n-n-n-n-tetramethyl-3-oxa-glutar-amide-tmoga-and-related-ligands-optical-properties-and-coordination-modes
#2
Lei Xu, Ning Pu, Taoxiang Sun, Youzhen Li, Pingping Wei, Jing Chen, Chao Xu
The complexation of hexavalent plutonyl Pu(vi) with N,N,N',N'-tetramethyl-3-oxa-glutar-amide (TMOGA) and its carboxylate analogs, N,N-dimethyl-3-oxa-glutaramic acid (DMOGA) and oxydiacetic acid (ODA), has been studied in 1.0 M NaClO4 by absorption spectrophotometry and density functional theory (DFT). Both 1 : 1 and 1 : 2 complexes of Pu(vi) with TMOGA, DMOGA and ODA have been identified and their stability constants were obtained and compared with those of hexavalent U(vi) and pentavalent Np(v). The resultant stability constants indicate that the ability of the three ligands to complex with Pu(vi) decreases in the order of ODA > DMOGA > TMOGA...
October 15, 2018: Dalton Transactions: An International Journal of Inorganic Chemistry
https://www.readbyqxmd.com/read/30315665/a-retrospective-study-of-157-hospitalized-cats-with-pancreatitis-in-a-tertiary-care-center-clinical-imaging-and-laboratory-findings-potential-prognostic-markers-and-outcome
#3
Ran Nivy, Alina Kaplanov, Sharon Kuzi, Michal Mazaki-Tovi, Einat Yas, Gilad Segev, Jennifer Ben-Oz, Eran Lavy, Itamar Aroch
BACKGROUND: Pancreatitis in cats (FP) has been increasingly diagnosed in recent years, but clinical studies of large numbers of affected cats are scarce. OBJECTIVES: To describe a large cohort of cats with FP requiring hospitalization. ANIMALS: One hundred and fifty-seven client-owned cats. METHODS: Retrospective study, including cats diagnosed with pancreatitis based on sonographic evidence, positive SNAP feline pancreatic lipase immunoreactivity test results, increased 1,2-o-dilauryl-rac-glycerol-glutaric Acid-(6'-methylresorufin ester)-lipase activity, histopathology, or some combination of these...
October 13, 2018: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/30311138/a-novel-truncating-flad1-variant-causing-multiple-acyl-coa-dehydrogenase-deficiency-madd-in-an-8-year-old-boy
#4
B Ryder, M Tolomeo, Z Nochi, M Colella, M Barile, R K Olsen, M Inbar-Feigenberg
Multiple acyl-CoA dehydrogenase deficiency (MADD) or glutaric aciduria type II (GAII) is a clinically heterogeneous disorder affecting fatty acid and amino acid metabolism. Presentations range from a severe neonatal form with hypoglycemia, metabolic acidosis, and hepatomegaly with or without congenital anomalies to later-onset lipid storage myopathy. Genetic testing for MADD traditionally comprises analysis of ETFA, ETFB, and ETFDH. Patients may respond to pharmacological doses of riboflavin, particularly those with late-onset MADD due to variants in ETFDH...
October 12, 2018: JIMD Reports
https://www.readbyqxmd.com/read/30298489/-analysis-of-gcdh-gene-mutations-in-3-patients-from-fujian-area-with-glutaric-academia-type-i
#5
Yao Chen, Qingying Lin, Yinglin Zeng, Hong Zhao, Weifen Chen, Jinfu Zhou, Yueqing Su, Feng Lin, Honghua Zhang, Wenbin Zhu
OBJECTIVE: To explore clinical features and mutation types in patients from Fujian area with glutaric academia type I(GA I). METHODS: Serum acylcarnitine and urine organic acid of 3 patients were determined with tandem mass spectrometry and gas chromatographic mass spectrometry. The patients also underwent magnetic resonance imaging analysis for the cranial region. Genomic DNA was extracted from peripheral blood samples, and the 12 exons and flanking regions of the GCDH gene were amplified with PCR and subjected to direct DNA sequencing...
October 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/30271473/glutaric-aciduria-type-1-with-microcephaly-masquerading-as-spastic-cerebral-palsy
#6
Indar K Sharawat, Lesa Dawman
Glutaric aciduria type 1 (GA-1) is an autosomal-recessive disorder caused by the deficiency of the mitochondrial enzyme glutaryl-CoA dehydrogenase. A 13-month-old boy presented with microcephaly, developmental delay, and progressive spasticity and was being treated as spastic cerebral palsy, later on had loss of developmental milestones after acute episode of illness at 12 months of age. The magnetic resonance imaging of brain revealed widened Sylvian fissure, hyperintensities in bilateral globus pallidus, and bilateral frontoparietal atrophy along with white matter loss...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271272/long-term-protein-packaging-in-bio-ionic-liquids-improved-catalytic-activity-and-enhanced-stability-of-cytochrome-c-against-multiple-stresses
#7
Meena Bisht, Dibyendu Mondal, Matheus M Pereira, Mara G Freire, P Venkatesu, J A P Coutinho
There is a considerable interest in the use of structurally stable and catalytically active enzymes, such as cytochrome C (Cyt C), in the pharmaceutical and fine chemical industries. However, harsh process conditions, such as temperature, pH, and presence of organic solvents, are the major barriers to the effective use of enzymes in biocatalysis. Herein, we demonstrate the suitability of bio-based ionic liquids (ILs) formed by the cholinium cation and dicarboxylate-based anions as potential media for enzymes, in which remarkable enhanced activity and improved stability of Cyt C against multiple stresses were obtained...
October 21, 2017: Green Chemistry: An International Journal and Green Chemistry Resource: GC
https://www.readbyqxmd.com/read/30218917/quantitation-of-plasma-and-urine-3-hydroxyglutaric-acid-after-separation-from-2-hydroxyglutaric-acid-and-other-compounds-of-similar-ion-transition-by-liquid-chromatography-tandem-mass-spectrometry-for-the-confirmation-of-glutaric-aciduria-type-1
#8
Garfield A Simon, Andrea Wierenga
BACKGROUND: Glutaric aciduria type 1, a deficiency of glutaryl-CoA dehydrogenase, causes an accumulation of neurotoxic metabolites glutaric acid and 3-hydroxyglutaric acid (3-HGA). Testing of these analytes is routinely done by GC-MS but seldom account for interference from isomers or compounds with similar ion transitions. We developed a liquid chromatography tandem mass spectrometry method that accurately measures 3-HGA in urine and plasma specimens, while utilizing similar reagents and instrumentation used for the routine performance of amino acid and acylcarnitine analysis in determining the diagnosis of several metabolic disorders...
October 15, 2018: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/30144560/metabolic-engineering-of-corynebacterium-glutamicum-for-the-production-of-glutaric-acid-a-c5-dicarboxylic-acid-platform-chemical
#9
Hee Taek Kim, Tae Uk Khang, Kei-Anne Baritugo, Sung Min Hyun, Kyoung Hee Kang, Sol Hee Jung, Bong Keun Song, Kyungmoon Park, Min-Kyu Oh, Gi Bae Kim, Hyun Uk Kim, Sang Yup Lee, Si Jae Park, Jeong Chan Joo
Corynebacterium glutamicum was metabolically engineered for the production of glutaric acid, a C5 dicarboxylic acid that can be used as platform building block chemicals including nylon and plasticizer. C. glutamicum gabT and gabD genes and Pseudomonas putida davT and davD genes encoding 5-aminovalerate transaminase and glutarate semialdehyde dehydrogenase, respectively, were examined in C. glutamicum for the construction of a glutaric acid bio-synthesis pathway along with P. putida davB and davA genes encoding lysine 2-monooxygenase and delta-aminovaleramidase, respectively...
August 22, 2018: Metabolic Engineering
https://www.readbyqxmd.com/read/30143200/production-of-glutaric-acid-from-5-aminovaleric-acid-using-escherichia-coli-whole-cell-bio-catalyst-overexpressing-gabtd-from-bacillus-subtilis
#10
Yun-Gi Hong, Yu-Mi Moon, Ju-Won Hong, So-Young No, Tae-Rim Choi, Hye-Rim Jung, Soo-Yeon Yang, Shashi Kant Bhatia, Jung-Oh Ahn, Kyung-Moon Park, Yung-Hun Yang
Glutaric acid is one of the promising C5 platform compounds in the biochemical industry. It can be produced chemically, through the ring-opening of butyrolactone followed by hydrolysis. Alternatively, glutaric acid can be produced via lysine degradation pathways by microorganisms. In microorganisms, the overexpression of enzymes involved in this pathway from E. coli and C. glutamicum has resulted in high accumulation of 5-aminovaleric acid. However, the conversion from 5-aminovaleric acid to glutaric acid has resulted in a relatively low conversion yield for unknown reasons...
November 2018: Enzyme and Microbial Technology
https://www.readbyqxmd.com/read/30129250/oxidative-damage-in-glutaric-aciduria-type-i-patients-and-the-protective-effects-of-l-carnitine-treatment
#11
Gilian Guerreiro, Jéssica Faverzani, Carlos Eduardo Diaz Jacques, Desirèe P Marchetti, Angela Sitta, Daniella de Moura Coelho, Aline Kayser, Fernando Kok, Larissa Athayde, Vanusa Manfredini, Moacir Wajner, Carmen Regla Vargas
The deficiency of the enzyme glutaryl-CoA dehydrogenase, known as glutaric acidemia type I (GA-I), leads to the accumulation of glutaric acid (GA) and glutarilcarnitine (C5DC) in the tissues and body fluids, unleashing important neurotoxic effects. l-carnitine (l-car) is recommended for the treatment of GA-I, aiming to induce the excretion of toxic metabolites. l-car has also demonstrated an important role as antioxidant and anti-inflammatory in some neurometabolic diseases. This study evaluated GA-I patients at diagnosis moment and treated the oxidative damage to lipids, proteins, and the inflammatory profile, as well as in vivo and in vitro DNA damage, reactive nitrogen species (RNS), and antioxidant capacity, verifying if the actual treatment with l-car (100 mg kg-1  day-1 ) is able to protect the organism against these processes...
August 20, 2018: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/30119976/selective-screening-of-fatty-acids-oxidation-defects-and-organic-acidemias-by-liquid-chromatography-tandem-mass-spectrometry-acylcarnitine-analysis-in-brazilian-patients
#12
Carmen Regla Vargas, Graziela Schmitt Ribas, Janine Machado da Silva, Angela Sitta, Marion Deon, Daniella de Moura Coelho, Moacir Wajner
BACKGROUND: Inborn errors of metabolism (IEM) are diseases which can lead to accumulation of toxic metabolites in the organism. AIM OF THE STUDY: To investigate, by selective screening, mitochondrial fatty acid oxidation defects (FAOD) and organic acidemias in Brazilian individuals with clinical suspicion of IEM. METHODS: A total of 7,268 individuals, from different regions of Brazil, had whole blood samples impregnated on filter paper which were submitted to the acylcarnitines analysis by liquid chromatography/tandem mass spectrometry (LC/MS/MS) at the Medical Genetics Service of Hospital de Clínicas de Porto Alegre, Brazil, during July 2008-July 2016...
April 2018: Archives of Medical Research
https://www.readbyqxmd.com/read/30115861/the-effect-of-plasma-pretreatment-and-cross-linking-degree-on-the-physical-and-antimicrobial-properties-of-nisin-coated-pva-films
#13
Zuzana Kolarova Raskova, Pavel Stahel, Jana Sedlarikova, Lenka Musilova, Monika Stupavska, Marian Lehocky
Stable antimicrobial nisin layers were prepared on the carrying medium-polyvinyl alcohol (PVA) films, crosslinked by glutaric acid. Surface plasma dielectric coplanar surface barrier discharge (DCSBD) modification of polyvinyl alcohol was used to improve the hydrophilic properties and to provide better adhesion of biologically active peptide-nisin to the polymer. The surface modification of films was studied in correlation to their cross-linking degree. Nisin was attached directly from the salt solution of the commercial product...
August 16, 2018: Materials
https://www.readbyqxmd.com/read/30110610/metabolic-and-immunological-responses-of-male-and-female-new-zealand-greenshell%C3%A2-mussels-perna-canaliculus-infected-with-vibrio-sp
#14
Thao V Nguyen, Andrea C Alfaro, Fabrice Merien, Tim Young, Roffi Grandiosa
Massive mortalities due to pathogens are routinely reported in bivalve cultivation that have significant economic consequences for the global aquaculture industry. However, host-pathogen interactions and infection mechanisms that mediate these interactions are poorly understood. In addition, gender-specific immunological responses have been reported for some species, but the reasons for such differences have not been elucidated. In this study, we used a GC/MS-based metabolomics platform and flow cytometry approach to characterize metabolic and immunological responses in haemolymph of male and female mussels (Perna canaliculus) experimentally infected with Vibrio sp...
September 2018: Journal of Invertebrate Pathology
https://www.readbyqxmd.com/read/30095186/global-metabolomic-responses-in-urine-from-atm-deficient-mice-in-response-to-ld-50-30-gamma-irradiation-doses
#15
Evagelia C Laiakis, Tytus D Mak, Steven J Strawn, Yi-Wen Wang, Bo-Hyun Moon, Pelagie Ake, Albert J Fornace
Exposures to ionizing radiation (IR) may either be accidental or intentional, for medical purposes or even through terrorist actions. As certain populations emerge to be more radiosensitive than others, it is imperative to assess those individuals and treat them accordingly. To demonstrate the feasibility of rapid identification of such cases, we utilized the highly radiosensitive mouse model Atm-/- in the C57BL/6 background, and evaluated the urinary responses in 8-10 week old male mice at early time points (4, 24, and 72 h) after exposure to their respective LD50/30 doses [4 Gy for Atm-/- , and 8 Gy for wild type (WT)]...
August 2018: Environmental and Molecular Mutagenesis
https://www.readbyqxmd.com/read/29996001/the-chemical-compositions-of-angelica-pubescens-oil-and-its-prevention-of-uv-b-radiation-induced-cutaneous-photoaging
#16
Dingkang Chen, Zhiyun Du, Zhirong Lin, Ping Su, Hanyi Huang, Zhirong Ou, Wanyi Pan, Shiya Huang, Kun Zhang, Xi Zheng, Li Lin, Lanyue Zhang
Angelica pubescens, a plant of the family Umbelliferae, has been widely used as traditional Chinese medicine for the treatment of many diseases. However, there has been minimal modern research focused on the pharmacological activity of oils extracted from Angelica pubescens, in particular, the potential anti-photoaging effects. Therefore, in the present study, we analyzed the chemical composition of Angelica pubescens oil (AO) and evaluated its bioactivity against photoaging in ultraviolet (UV) -B radiation-induced hairless mice...
July 11, 2018: Chemistry & Biodiversity
https://www.readbyqxmd.com/read/29962642/anesthetic-management-for-fracture-head-of-radius-in-a-child-with-glutaric-aciduria-type-1
#17
Nandita Mehta, Gurleen Kaur
Glutaric aciduria Type 1 (GA-1) is an autosomal recessive metabolic disorder that results from deficiency of enzyme glutaryl-CoA dehydrogenase. This gives rise to elevated neurotoxic glutaric acid and 3-hydroxyglutaric acid as well as nontoxic glutarylcarnitine in body fluids. The enzyme defect leads to secondary damage to central nervous system due to the accumulation of glutaric acid. Approximately 90% people will develop the neurological disease during a finite period of brain development (3-36 months) following an acute encephalopathic crisis often precipitated by gastroenteritis, immunization, surgical intervention, and intercurrent febrile illness...
April 2018: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/29954488/glutaryl-polyamidoamine-dendrimer-for-overcoming-cisplatin-resistance-of-breast-cancer-cells
#18
Xiaoping Xu, Yi Li, Xiaoling Lu, Yuyun Sun, Jianming Luo, Yingjian Zhang
OBJECTIVE: Cisplatin has limited clinical applications due to drug resistance. PAMAM dendrimer was chosen as a vehicle to counteract cisplatin-resistance and its mechanism was assessed. METHODS: Generation 5 Polyamidoamine dendrimer (G5) was modified by glutaric anhydride (GA) and then conjugated with cisplatin. The cisplatin release of G5-GA-cisplatin was evaluated at pH 5.5 and pH 7.4. The cytotoxicity of G5-GA-cisplatin and free cisplatin was compared in cisplatin-resistant breast cancer cell line MCF-7R...
October 1, 2018: Journal of Nanoscience and Nanotechnology
https://www.readbyqxmd.com/read/29947519/challenges-in-transitioning-cocrystals-from-bench-to-bedside-dissociation-in-prototype-drug-product-environment
#19
Sampada Koranne, Anasuya Sahoo, Joseph F Krzyzaniak, Suman Luthra, Kapildev K Arora, Raj Suryanarayanan
Tablets containing a theophylline-glutaric acid (TG) cocrystal dissociated rapidly forming crystalline theophylline (20-30%), following storage at 40 °C/75% RH for 2 weeks. Control tablets of TG cocrystal containing no excipients were stable under the same conditions. The dissociation reaction was water-mediated, and the theophylline concentration (the dissociation product), monitored by synchrotron X-ray diffractometry, was strongly influenced by the formulation composition. Investigation of the binary compacts of the TG cocrystal with each excipient revealed the influence of excipient properties (hydrophilicity, ionizability) on cocrystal stability, providing mechanistic insights into a dissociation reaction...
August 6, 2018: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/29940867/a-case-series-evaluating-the-impact-of-hepatitis-c-eradication-using-direct-acting-antivirals-on-primary-biliary-cholangitis-associated-autoimmunity
#20
Henry H Nguyen, Abdullah Khathlan, Marvin J Fritzler, Mark G Swain
BACKGROUND: Chronic Hepatitis C Virus (HCV) infection has been commonly linked to the development of autoimmunity, in part through activation of B cells. B cells are also postulated to play a pathogenic role in the autoimmune liver disease Primary Biliary Cholangitis (PBC). Patients with concurrent PBC and HCV infection carry an increased risk of more progressive disease, although the mechanism underlying this effect is poorly understood. Utilizing a case series of patients with concurrent PBC and HCV, the aim of this study was to evaluate for the potential impact of HCV eradication upon autoimmunity/autoantibody production...
June 25, 2018: BMC Gastroenterology
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