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Pyridoxine

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https://www.readbyqxmd.com/read/29142798/verification-of-diagnosis-in-tuberculosis-a-case-report-and-discussion
#1
Amanda M Dave, Abed Adelrahman, Vishist Mehta, Stephen Cavalieri, Renuga Vivekanadan
Tuberculosis (TB), caused by strains of Mycobacterium tuberculosis complex (M. tuberculosis), is a pulmonary infection that is spread by airborne droplet transmission. The development and spread of drug-resistant strains of M. tuberculosisgreatly jeopardize TB control efforts. We report the case of a previously healthy 43-year-old male, visiting from China, who presented to the emergency department complaining of hemoptysis of 10 days' duration. Cultures were positive for acid fast bacteria and negative for fungi...
September 3, 2017: Curēus
https://www.readbyqxmd.com/read/29119254/potential-pharmacological-chaperones-for-cystathionine-beta-synthase-deficient-homocystinuria
#2
Tomas Majtan, Angel L Pey, Paula Gimenez-Mascarell, Luis Alfonso Martínez-Cruz, Csaba Szabo, Viktor Kožich, Jan P Kraus
Classical homocystinuria (HCU) is the most common loss-of-function inborn error of sulfur amino acid metabolism. HCU is caused by a deficiency in enzymatic degradation of homocysteine, a toxic intermediate of methionine transformation to cysteine, chiefly due to missense mutations in the cystathionine beta-synthase (CBS) gene. As with many other inherited disorders, the pathogenic mutations do not target key catalytic residues, but rather introduce structural perturbations leading to an enhanced tendency of the mutant CBS to misfold and either to form nonfunctional aggregates or to undergo proteasome-dependent degradation...
November 10, 2017: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/29105823/pyridoxine-sensitive-x-linked-sideroblastic-anaemia-in-the-absence-of-ring-sideroblasts-molecular-diagnosis
#3
Thomas Creasey, Tina Biss, John Lambert, Frances Smith, Barnaby Clark, Peter Carey
No abstract text is available yet for this article.
November 5, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29080399/pyridoxine-responsiveness-in-pyridox-am-ine-5-phosphate-oxidase-deficiency-the-importance-of-early-treatment
#4
Patrizia Accorsi, Elena Cellini, Claudia Di Paolantonio, Gianvito Panzarino, Alberto Verrotti, Lucio Giordano
No abstract text is available yet for this article.
October 23, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/29071784/simultaneous-determination-of-niacin-and-pyridoxine-at-trace-levels-by-using-diode-array-high-performance-liquid-chromatography-and-liquid-chromatography-with-quadrupole-time-of-flight-tandem-mass-spectrometry
#5
Sabriye Sel, Elif Öztürk Er, Sezgin Bakırdere
A highly sensitive and simple diode-array high-performance liquid chromatography and liquid chromatography with quadrupole time-of-flight tandem mass spectrometry method was developed for the simultaneous determination of niacin and pyridoxine in pharmaceutical drugs, tap water and wastewater samples. To determine the in vivo behavior of niacin and pyridoxine, analytes were subjected to simulated gastric conditions. The calibration plots of the diode-array high-performance liquid chromatography and liquid chromatography with quadrupole time-of-flight tandem mass spectrometry method showed good linearity over a wide concentration range with close to 1...
October 26, 2017: Journal of Separation Science
https://www.readbyqxmd.com/read/29061647/pyridoxine-dependent-epilepsy-in-zebrafish-caused-by-aldh7a1-deficiency
#6
Izabella A Pena, Yann Roussel, Kate Daniel, Kevin Mongeon, Devon Johnstone, Hellen Weinschutz Mendes, Marjolein Bosma, Vishal Saxena, Nathalie Lepage, Pranesh Chakraborty, David A Dyment, Clara D M van Karnebeek, Nanda Verhoeven-Duif, Tuan Vu Bui, Kym M Boycott, Marc Ekker, Alex MacKenzie
Pyridoxine-dependent epilepsy (PDE) is a rare disease characterized by mutations in the lysine degradation gene ALDH7A1 leading to recurrent neonatal seizures which are uniquely alleviated by high doses of pyridoxine or pyridoxal 5'-phosphate (vitamin B6 vitamers). Despite treatment, neurodevelopmental disabilities are still observed in most PDE patients underlining the need for adjunct therapies. Over 60 years after the initial description of PDE, we report the first animal model for this disease: an aldh7a1-null zebrafish (Danio rerio) displaying deficient lysine metabolism and spontaneous and recurrent seizures in the larval stage (10 dpf)...
October 23, 2017: Genetics
https://www.readbyqxmd.com/read/29057185/combination-of-pyridoxine-and-thiamine-treatment-in-bilateral-induced-ptosis-in-a-child-case-report-and-review-of-literature
#7
Khai-Siang Chai, Mohamad Norsarwany, Ismail Shatriah
Ptosis is a rare side effect of vincristine chemotherapy in patients treated for cancer. We report a case of a child with common B-cell acute lymphoblastic leukemia who developed bilateral moderate ptosis following the chemotherapy protocol of the United Kingdom Acute Lymphoblastic Leukemia (ALL) regimen A. The patient showed dramatic clinical improvement after a combination of oral pyridoxine and thiamine treatment. We provide a literature review of this uncommon presentation.
August 16, 2017: Curēus
https://www.readbyqxmd.com/read/29053735/characterization-of-the-first-knock-out-aldh7a1-zebrafish-model-for-pyridoxine-dependent-epilepsy-using-crispr-cas9-technology
#8
Nikita Zabinyakov, Garrett Bullivant, Feng Cao, Matilde Fernandez Ojeda, Zheng Ping Jia, Xiao-Yan Wen, James J Dowling, Gajja S Salomons, Saadet Mercimek-Andrews
Pyridoxine dependent epilepsy (PDE) is caused by likely pathogenic variants in ALDH7A1 (PDE-ALDH7A1) and inherited autosomal recessively. Neurotoxic alpha-amino adipic semialdehyde (alpha-AASA), piperideine 6-carboxylate and pipecolic acid accumulate in body fluids. Neonatal or infantile onset seizures refractory to anti-epileptic medications are clinical features. Treatment with pyridoxine, arginine and lysine-restricted diet does not normalize neurodevelopmental outcome or accumulation of neurotoxic metabolites...
2017: PloS One
https://www.readbyqxmd.com/read/29053132/-neurotropic-effect-of-b-vitamins-in-the-complex-treatment-of-pain-syndrome
#9
O A Shavlovskaya
Combined vitamin preparations in therapeutic doses are used, along with simple analgesics, nonsteroidal anti-inflammatory drugs (NSAIDs), muscle and epidural blockade, for the relief of acute pain. It is recommended to use the B vitamin preparation neuromultivit. The tablet form of this preparation contains vitamin thiamine hydrochloride (100 mg), pyridoxine hydrochloride (200 mg), cyanocobalamin (0.2 mg), and injectable form includes thiamine hydrochloride (100mg), pyridoxine hydrochloride (100 mg), cyanocobalamin (1 mg)...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/29045138/importance-of-the-c-terminus-of-aldehyde-dehydrogenase-7a1-for-oligomerization-and-catalytic-activity
#10
David A Korasick, Jesse W Wyatt, Min Luo, Adrian R Laciak, Kasi Ruddraraju, Kent S Gates, Michael T Henzl, John J Tanner
Aldehyde dehydrogenase 7A1 (ALDH7A1) catalyzes the terminal step of lysine catabolism, the NAD(+)-dependent oxidation of α-aminoadipate semialdehyde to α-aminoadipate. Structures of ALDH7A1 reveal the C-terminus is a gate that opens and closes in response to the binding of α-aminoadipate. In the closed state, the C-terminus of one protomer stabilizes the active site of the neighboring protomer in the dimer-of-dimers tetramer. Specifically, Ala505 and Gln506 interact with the conserved aldehyde anchor loop structure in the closed state...
October 25, 2017: Biochemistry
https://www.readbyqxmd.com/read/29043603/epigenetic-modifications-of-gene-expression-by-lifestyle-and-environment
#11
REVIEW
Qudeer Ahmed Abdul, Byung Pal Yu, Hae Young Chung, Hyun Ah Jung, Jae Sue Choi
Epigenetics oftenly described as the heritable changes in gene expression independent of changes in DNA sequence. Various environmental factors such as nutrition-dietary components, lifestyle, exercise, physical activity, toxins, and other contributing factors remodel the genome either in a constructive or detrimental way. Since epigenetic changes are reversible and nutrition is one of the many epigenetic regulators that modify gene expression without changing the DNA sequence, dietary nutrients and bioactive food components contribute to epigenetic phenomena either by directly suppressing DNA methylation or histone catalyzing enzymes or by changing the availability of substrates required for enzymatic reactions...
October 17, 2017: Archives of Pharmacal Research
https://www.readbyqxmd.com/read/29034851/nitrosopumilus-maritimus-gen-nov-sp-nov-nitrosopumilus-cobalaminigenes-sp-nov-nitrosopumilus-oxyclinae-sp-nov-and-nitrosopumilus-ureiphilus-sp-nov-four-marine-ammonia-oxidizing-archaea-of-the-phylum-thaumarchaeota
#12
Wei Qin, Katherine R Heal, Rasika Ramdasi, Julia N Kobelt, Willm Martens-Habbena, Anthony D Bertagnolli, Shady A Amin, Christopher B Walker, Hidetoshi Urakawa, Martin Könneke, Allan H Devol, James W Moffett, E Virginia Armbrust, Grant J Jensen, Anitra E Ingalls, David A Stahl
Four mesophilic, neutrophilic, and aerobic marine ammonia-oxidizing archaea, designated strains SCM1(T), HCA1(T), HCE1(T) and PS0(T), were isolated from a tropical marine fish tank, dimly lit deep coastal waters, the lower euphotic zone of coastal waters, and near-surface sediment in the Puget Sound estuary, respectively. Cells are straight or slightly curved small rods, 0.15-0.26 µm in diameter and 0.50-1.59 µm in length. Motility was not observed, although strain PS0(T) possesses genes associated with archaeal flagella and chemotaxis, suggesting it may be motile under some conditions...
October 16, 2017: International Journal of Systematic and Evolutionary Microbiology
https://www.readbyqxmd.com/read/28985901/systemic-manifestations-in-pyridox-am-ine-5-phosphate-oxidase-deficiency
#13
Réjean M Guerriero, Archana A Patel, Brian Walsh, Fiona M Baumer, Ankoor S Shah, Jurriaan M Peters, Lance H Rodan, Pankaj B Agrawal, Phillip L Pearl, Masanori Takeoka
OBJECTIVE: Pyridoxine is converted to its biologically active form pyridoxal-5-phosphate (P5P) by the enzyme pyridox(am)ine 5'-phosphate oxidase and serves as a cofactor in nearly 200 reactions in the central nervous system. Pyridox(am)ine 5'-phosphate oxidase deficiency leads to P5P dependent epilepsy, typically a neonatal- or infantile-onset epileptic encephalopathy treatable with P5P or in some cases, pyridoxine. Following identification of retinopathy in a patient with pyridox(am)ine 5'-phosphate oxidase deficiency that was reversible with P5P therapy, we describe the systemic manifestations of pyridox(am)ine 5'-phosphate oxidase deficiency...
June 3, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28980096/isolated-aortic-root-dilation-in-homocystinuria
#14
Massimiliano Lorenzini, Nishan Guha, James E Davison, Alex Pitcher, Bejal Pandya, Helena Kemp, Robin Lachmann, Perry M Elliott, Elaine Murphy
BACKGROUND: Vascular complications in homocystinuria have been known for many years, but there have been no reports to date on involvement of the ascending aorta. METHODS: We conducted a cross-sectional study of patients with homocystinuria, known to a single metabolic centre, and evaluated in 2016 with a transthoracic echocardiogram. Aortic root dilation was defined as Z-score ≥ 2.0 SD, and graded mild (Z-score 2.0-3.0), moderate (Z-score 3.01-4.0) and severe (Z-score > 4...
October 4, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28962114/clinical-and-genetic-characteristics-of-pyridoxine-dependent-epilepsy-case-series-report-of-three-chinese-patients-with-phenotypic-variability
#15
Sanmei Wang, Jing Sun, Yao Tu, Lina Zhu, Zhichun Feng
Pyridoxine-dependent epilepsy (PDE) is a rare disorder caused by aldehyde dehydrogenase 7 family member A1 (ALDH7A1) deficiency. The present study reported on three Chinese cases of PDE with phenotypic variability for providing further insight into this disease. All three patients presented with recurrent seizures and readily responded to treatment with pyridoxine, in line with the typical symptomology of PDE. The three cases varied in their clinical manifestations with regard to the time of onset, seizure type, EEG findings and mental development...
September 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28951222/dietary-pyridoxine-deficiency-reduced-growth-performance-and-impaired-intestinal-immune-function-associated-with-tor-and-nf-%C3%AE%C2%BAb-signalling-of-young-grass-carp-ctenopharyngodon-idella
#16
Xin Zheng, Lin Feng, Wei-Dan Jiang, Pei Wu, Yang Liu, Jun Jiang, Sheng-Yao Kuang, Ling Tang, Wu-Neng Tang, Yong-An Zhang, Xiao-Qiu Zhou
The objective of this study was to evaluate the effects of dietary pyridoxine (PN) deficiency on growth performance, intestinal immune function and the potential regulation mechanisms in young grass carp (Ctenopharyngodon idella). Fish were fed six diets containing graded levels of PN (0.12-7.48 mg/kg) for 70 days. After that, a challenge test was conducted by infection of Aeromonas hydrophila for 14 days. The results showed that compared with the optimal PN level, PN deficiency: (1) reduced the production of innate immune components such as lysozyme (LZ), acid phosphatase (ACP), complements and antimicrobial peptides and adaptive immune components such as immunoglobulins in three intestinal segments of young grass carp (P < 0...
September 23, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/28935605/pyridoxine-improves-hippocampal-cognitive-function-via-increases-of-serotonin-turnover-and-tyrosine-hydroxylase-and-its-association-with-cb1-cannabinoid-receptor-interacting-protein-and-the-cb1-cannabinoid-receptor-pathway
#17
Hyo Young Jung, Dae Won Kim, Sung Min Nam, Jong Whi Kim, Jin Young Chung, Moo-Ho Won, Je Kyung Seong, Yeo Sung Yoon, Dae Young Yoo, In Koo Hwang
BACKGROUND: In the present study, we investigated the effects of pyridoxine on hippocampal functions and changes in protein profiles based on the proteomic approach. METHODS: Eight-week-old mice received intraperitoneal injections of physiological saline (vehicle) or 350mg/kg pyridoxine twice a day for 21days. RESULTS: Phosphoglycerate mutase 1 was up-regulated, while CB1 cannabinoid receptor-interacting protein 1 (CRIP1) was down-regulated, in the pyridoxine-treated group...
December 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28912874/dietary-vitamin-b6-modulates-the-gene-expression-of-myokines-nrf2-related-factors-myogenin-and-hsp60-in-the-skeletal-muscle-of-rats
#18
Sofya Suidasari, Shinji Uragami, Noriyuki Yanaka, Norihisa Kato
Previous studies have suggested that vitamin B6 is an ergogenic factor. However, the role of dietary vitamin B6 in skeletal muscle has not been widely researched. The aim of the present study was to investigate the effects of dietary vitamin B6 on the gene expression of 19 myokines, 14 nuclear factor erythroid 2-related factor 2 (Nrf2)-regulated factors, 8 myogenesis-related factors and 4 heat shock proteins (HSPs), which may serve important roles in skeletal muscles. Rats were fed a diet containing 1 (marginal vitamin B6 deficiency), 7 (recommended dietary level) or 35 mg/kg of pyridoxine (PN) HCl/ for 6 weeks...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28904440/primary-hyperoxaluria-type-1-with-homozygosity-for-a-double-mutated-agxt-allele-in-a-2-year-old-child
#19
S Krishnamurthy, G B Kartha, V S Venkateswaran, M Prasannakumar, S Mahadevan, M Gowda, A Pelle, D Giachino
Primary hyperoxaluria (PH) Type 1 is a rare, genetic disorder caused by deficiency of the liver enzyme alanine-glyoxylate aminotransferase, which is encoded by AGXT gene. We report a 2-year-old South Indian Tamil child with nephrocalcinosis due to PH Type 1, in whom a homozygous genotype for two missense mutations in the AGXT gene was found: first, a C to G transversion (c. 32C>G) in exon 1 resulting in the amino acid substitution p.Pro11Arg; second, a T to A transversion (c. 167T>A) in exon 2 resulting in p...
September 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28877263/the-transcriptional-landscape-of-rhizoctonia-solani-ag1-ia-during-infection-of-soybean-as-defined-by-rna-seq
#20
Tanya R Copley, Raj Duggavathi, Suha Jabaji
Rhizoctonia solani Kühn infects most plant families and can cause significant agricultural yield losses worldwide; however, plant resistance to this disease is rare and short-lived, and therefore poorly understood, resulting in the use of chemical pesticides for its control. Understanding the functional responses of this pathogen during host infection can help elucidate the molecular mechanisms that are necessary for successful host invasion. Using the pathosystem model soybean-R. solani anastomosis group AG1-IA, we examined the global transcriptional responses of R...
2017: PloS One
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