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Glutaric Acid

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https://www.readbyqxmd.com/read/30510944/glutaric-acidemia-type-ii-patient-with-thalassemia-minor-and-novel-electron-transfer-flavoprotein-a-gene-mutations-a-case-report-and-review-of-literature
#1
Neslihan Yildirim Saral, Fehime Benli Aksungar, Cigdem Aktuglu-Zeybek, Julide Coskun, Ozlem Demirelce, Mustafa Serteser
Glutaric acidemia type II (GAII), also known as multiple acyl-CoA dehydrogenase deficiency, is an autosomal recessive inborn error of amino acid and fatty acid metabolism. We report a case of GAII with novel electron transfer flavoprotein (ETF)-A mutations in a 2-year-old female with thalassemia minor. The patient developed an episode of hypoglycemia and hypotonicity on the postnatal first day. Laboratory investigations revealed elevations of multiple acyl carnitines indicating glutaric acidemia type II in newborn screening analysis...
November 26, 2018: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/30483387/serum-based-metabolic-alterations-in-patients-with-papillary-thyroid-carcinoma-unveiled-by-non-targeted-1h-nmr-metabolomics-approach
#2
Reyhaneh Farrokhi Yekta, Mostafa Rezaei Tavirani, Afsaneh Arefi Oskouie, Mohammad Reza Mohajeri-Tehrani, Ahmad Reza Soroush, Alireza Akbarzadeh Baghban
Objectives: As the most prevalent endocrine system malignancy, papillary thyroid carcinoma had a very fast rising incidence in recent years for unknown reasons besides the fact that the current methods in thyroid cancer diagnosis still hold some limitations. Therefore, the aim of this study was to improve the potential molecular markers for diagnosis of benign and malignant thyroid nodules to prevent unnecessary surgeries for benign tumors. Materials and Methods: In this study, 1H-NMR metabolomics platform was used to seek the discriminating serum metabolites in malignant papillary thyroid carcinoma (PTC) compared to benign multinodular goiter (MNG) and healthy subjects and also to better understand the disease mechanisms using bioinformatics analysis...
November 2018: Iranian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/30477628/-reye-syndrome-and-sudden-death-symptoms-after-oral-administration-of-nimesulide-due-to-upper-respiratory-tract-infection-in-a-boy
#3
Li-Fang Feng, Xiao-Hong Chen, Dong-Xiao Li, Xi-Yuan Li, Jin-Qing Song, Ying Jin, Yan-Ling Yang
A boy aged 6 years and 3 months developed upper respiratory tract infection and pyrexia 2 months ago and was given oral administration of nimesulide by his parents according to directions. Half an hour later, the boy experienced convulsions and cardiopulmonary arrest, and emergency examination found hypoketotic hypoglycemia, metabolic acidosis, significant increases in serum aminotransferases and creatine kinase, and renal damage. Recovery of consciousness and vital signs was achieved after cardiopulmonary resuscitation, but severe mental and movement regression was observed...
November 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/30458104/conformation-dependent-coordination-of-carboxylic-acids-with-fe-3-o-4-nanoparticles-studied-by-atr-ftir-spectral-deconvolution
#4
Xiaorui Zhang, Lan Chen, Li Yuan, Renxiao Liu, Dexing Li, Xiao-Ping Liu, Guanglu Ge
The coordination of valeric acid (VA), glutaric acid (GA) and tricarballylic acid (TA) with Fe-OH on the Fe3 O4 nanoparticle surface has been systematically studied to elucidate the effect of COOH, molecular configuration and ligand concentration on the coordination by the combining use of attenuated total reflectance Fourier transform infrared (ATR-FTIR) and thermogravimetric analysis (TGA). The results show that the binding ability of the acids increases with the increase of the COOH number. Multiple conformations coexist for the dicarboxylic and tricarboxylic acid coordinated on the iron oxide NPs...
November 20, 2018: Langmuir: the ACS Journal of Surfaces and Colloids
https://www.readbyqxmd.com/read/30451603/two-cases-of-glutaric-aciduria-type-ii-how-to-differentiate-from-inflammatory-myopathies
#5
Meltem Koca, Abdulsamet Erden, Berkan Armagan, Alper Sari, Fatih Yildiz, Sevim Ozdamar, Umut Kalyoncu, Omer Karadag
Muscle weakness is a nonspecific finding of myopathy of any etiology that include iatrogenic, toxic, endocrinological, infectious, immunologic, and metabolic disorders. Among the metabolic myopathies glutaric aciduria type II (GAII) is an autosomal recessively inherited rare disorder of fatty acid and amino acid metabolisms. The late onset form is heterogeneous in terms of symptomatology and severity and for the cases that chronic manifestations of lipid storage myopathy are the only clues for the disease, differential diagnosis can be challenging...
November 19, 2018: Acta Clinica Belgica
https://www.readbyqxmd.com/read/30450795/enhanced-production-of-glutaric-acid-by-nadh-oxidase-and-gabd-reinforced-bioconversion-from-l-lysine
#6
Yoon Gi Hong, Yu-Mi Moon, Tae-Rim Choi, Hye-Rim Jung, Soo-Yeon Yang, Jung-Oh Ahn, Jeong-Chan Joo, Kyungmoon Park, Yun-Gon Kim, Shashi Kant Bhatia, Yoo Kyung Lee, Yung-Hun Yang
Glutaric acid is a promising alternative chemical to phthalate plasticizer, since it can be produced by the bioconversion of lysine. Though recent studies have enabled high-yield production of its precursor, 5-aminovaleric acid (AMV), glutaric acid production via the AMV pathway has been limited by need for cofactors. Introduction of NAD(P)H oxidase (Nox) with GabTD enzyme remarkably diminished the demand for NAD+ . Supply of oxygen through vigorous shaking had a significant effect on the conversion of AMV with a reduced requirement of NAD+ ...
November 18, 2018: Biotechnology and Bioengineering
https://www.readbyqxmd.com/read/30425699/efficient-production-of-the-dicarboxylic-acid-glutarate-by-corynebacterium-glutamicum-via-a-novel-synthetic-pathway
#7
Fernando Pérez-García, João M P Jorge, Annika Dreyszas, Joe Max Risse, Volker F Wendisch
The dicarboxylic acid glutarate is an important building-block gaining interest in the chemical and pharmaceutical industry. Here, a synthetic pathway for fermentative production of glutarate by the actinobacterium Corynebacterium glutamicum has been developed. The pathway does not require molecular oxygen and operates via lysine decarboyxylase followed by two transamination and two NAD-dependent oxidation reactions. Using a genome-streamlined L-lysine producing strain as basis, metabolic engineering was performed to enable conversion of L-lysine to glutarate in a five-step synthetic pathway comprising lysine decarboxylase, putrescine transaminase and γ-aminobutyraldehyde dehydrogenase from Escherichia coli and GABA/5AVA amino transferase and succinate/glutarate semialdehyde dehydrogenase either from C...
2018: Frontiers in Microbiology
https://www.readbyqxmd.com/read/30411057/iedda-conjugation-reaction-in-radiometal-labeling-of-peptides-with-68-ga-and-64-cu-unexpected-findings
#8
Shanna Litau, Uwe Seibold, Björn Wängler, Ralf Schirrmacher, Carmen Wängler
The inverse electron demand Diels-Alder conjugation reaction has gained increasing importance over the past few years for efficient in vivo and ex vivo radiometal labeling of antibodies. However, the application of this very fast reaction type has not been studied for radiolabeling of peptides so far. We show here the synthesis of 3-benzyl-1,2,4,5-tetrazine-comprising ((1,4,7,10-tetraazacyclododecane-4,7,10-triyl)triacetic acid-1-glutaric acid) (DOTA-GA) and ((1,4,7-triazacyclononane-4,7-diyl)diacetic acid-1-glutaric acid) (NODA-GA) chelators and their radiometal labeling with 68 Ga3+ and 64 Cu2+ ...
October 31, 2018: ACS Omega
https://www.readbyqxmd.com/read/30340205/hygroscopic-behavior-and-fractional-crystallization-of-mixed-nh-4-2-so-4-glutaric-acid-aerosols-by-vacuum-ftir
#9
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...
February 5, 2019: 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
#10
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 30, 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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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 chemical for nylons and plasticizers. 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 biosynthesis pathway along with P. putida davB and davA genes encoding lysine 2-monooxygenase and delta-aminovaleramidase, respectively...
August 23, 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
#18
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
#19
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
#20
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
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