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Dong-Xiao Li, Xi-Yuan Li, Hui Dong, Yu-Peng Liu, Yuan Ding, Jin-Qing Song, Ying Jin, Yao Zhang, Qiao Wang, Yan-Ling Yang
BACKGROUND: Classical homocystinuria (homocysteinemia type 1, MIM# 236200) is a rare inherited disorder in Mainland China. This study aimed to identify mutations in the cystathionine β-synthase (CBS) gene which are associated with classical homocystinuria in nine Chinese patients. METHODS: Nine Chinese patients were diagnosed at the age of 5 years 4 months to 18 years by plasma total homocysteine and blood methionine determination. CBS gene analysis was performed for the patients and their families...
March 5, 2018: World Journal of Pediatrics: WJP
Hee Seon Lim, Dong-Ho Seo, In-Tae Cha, Hyunjin Lee, Young-Do Nam, Myung-Ji Seo
A putative gene (gadlbhye1) encoding glutamate decarboxylase (GAD) was cloned from Lactobacillus brevis HYE1 isolated from kimchi, a traditional Korean fermented vegetable. The amino acid sequences of GADLbHYE1 showed 48% homology with the GadA family and 99% identity with the GadB family from L. brevis. The cloned GADLbHYE1 was functionally expressed in Escherichia coli using inducible expression vectors. The expressed recombinant GADLbHYE1 was successfully purified by Ni-NTA affinity chromatography, and had a molecular mass of 54 kDa with optimal hydrolysis activity at 55 °C and pH 4...
March 2, 2018: World Journal of Microbiology & Biotechnology
Hyuk Hwa Kim, Yu-Ri Kang, Jung-Yun Lee, Hung-Bae Chang, Ki Won Lee, Emmanouil Apostolidis, Young-In Kwon
In the current study, we investigated the inhibitory activity of pyridoxine, pyridoxal, and pyridoxamine, against various digestive enzymes such as α-glucosidases, sucrase, maltase, and glucoamylase. Inhibition of these enzymes involved in the absorption of disaccharide can improve post-prandial hyperglycemia due to a carbohydrate-based diet. Pyridoxal (4.14 mg/mL of IC50 ) had the highest rat intestinal α-glucosidase inhibitory activity, followed by pyridoxamine and pyridoxine (4.85 and 5.02 mg/mL of IC50 , respectively)...
February 28, 2018: Nutrients
Serkan Kırık, Olcay Güngör, Sedat Işıkay, Can Acıpayam, Yasemin Çoban, Emre Çelik
Decreased height of the eyelid or the narrowing of the lid is called ptosis. Ptosis has several causes. Malignancy-related conditions such as Horner's syndrome, which causes unilateral ptosis in the pediatric age group, and patients with malignancy receiving chemotherapeutic treatment, are often secondary to these drugs and ptosis is a clue of underlying diseases. Underlying pathologies can lead to different clinical conditions such as cognitive impairment from coma, the presence of ptosis should be cautionary...
December 2017: Türk Pediatri Arşivi
D Thindwa, P MacPherson, A T Choko, M Khundi, R Sambakunsi, L G Ngwira, T Kalua, E L Webb, E L Corbett
SETTING: Despite worldwide scale-up of human immunodeficiency virus (HIV) care services, relatively few countries have implemented isoniazid preventive therapy (IPT). Among other programmatic concerns, IPT completion tends to be low, especially when not fully integrated into HIV care clinics. OBJECTIVE: To estimate non-completion of 6-month IPT and its predictors among HIV-positive adults aged 16 years. DESIGN: A prospective cohort study nested within a cluster-randomised trial of TB prevention was conducted between February 2012 and June 2014...
March 1, 2018: International Journal of Tuberculosis and Lung Disease
Plamen Katsarov, Georgi Gergov, Aylin Alin, Bissera Pilicheva, Yahya Al-Degs, Vasil Simeonov, Margarita Kassarova
The prediction power of partial least squares (PLS) and multivariate curve resolution-alternating least squares (MCR-ALS) methods have been studied for simultaneous quantitative analysis of the binary drug combination - doxylamine succinate and pyridoxine hydrochloride. Analysis of first-order UV overlapped spectra was performed using different PLS models - classical PLS1 and PLS2 as well as partial robust M-regression (PRM). These linear models were compared to MCR-ALS with equality and correlation constraints (MCR-ALS-CC)...
March 1, 2018: Acta Pharmaceutica
Silvia Marino, Giovanna Vitaliti, Simona Domenica Marino, Piero Pavone, Simona Provvidenti, Catia Romano, Raffaele Falsaperla
BACKGROUND: Few studies on adult and pediatric patients have shown pyridoxine efficacy as additional therapy for those receiving levetiracetam (LEV) to prevent and mitigate behavioral adverse effects (BAEs). OBJECTIVE: The aim of our study was to analyze the safety and efficacy of pyridoxine supplementation in the prevention of LEV adverse effects, including suicidal ideation. METHODS: This randomized, case-control trial included patients receiving LEV as monotherapy treatment...
February 1, 2018: Annals of Pharmacotherapy
Taehyun Roh, Umasankar De, Seong Kwang Lim, Min Kook Kim, Seul Min Choi, Duck Soo Lim, Sungpil Yoon, Sam Kacew, Hyung Sik Kim, Byung-Mu Lee
The detoxifying effect of pyridoxine against acetaminophen (APAP)-induced hepatotoxicity was investigated. HepG2 cells were co-treated with APAP and pyridoxine to compare with betaine or methionine for 24 h. LDH, ALT and AST activities were measured to determine direct cells damage in vitro and in vivo. Lipid peroxidation, antioxidant enzymes activity, and glutathione level were measured. Cytochrome c releaseand procaspase-3, cleaved caspase-3, Bcl-2, or Bax protein levels were measured to determine APAP-induced apoptotic cell death...
February 10, 2018: Food and Chemical Toxicology
Marie-Coralie Cornet, Tristan T Sands, Maria Roberta Cilio
Whereas the majority of seizures in neonates are related to acute brain injury, a substantial minority are the first symptom of a neonatal-onset epilepsy, often linked to a pathogenic genetic variant. This defect may disrupt cortical development (e.g., lissencephaly, focal cortical dysplasia), lead to metabolic changes (e.g., pyridoxine-dependent epilepsy, sulfite oxidase deficiency) or lead to cortical dysfunction without metabolic or macroscopic structural changes (e.g., channelopathies, STXBP1). Historically, studies on treatment response and long-term consequences of neonatal seizures have lumped all etiologies together...
January 31, 2018: Seminars in Fetal & Neonatal Medicine
K L Westmacott, A Crew, O Doran, J P Hart
This paper describes the development of a novel electrochemical assay for the measurement of water-soluble vitamins in food and pharmaceutical products. The optimum conditions for the determination of vitamin B 1 (thiamine), B 2 (riboflavin) and B 6 (pyridoxine) in phosphate buffer were established using cyclic voltammetry in conjunction with screen printed carbon electrodes (SPCEs). The optimum current response for all three vitamins was achieved in 0.1M phosphate buffer pH 11 using an initial potential of -1...
May 1, 2018: Talanta
Kristina M Brooks, Jomy M George, Alice K Pau, Adam Rupert, Carolina Mehaffy, Prithwiraj De, Karen M Dobos, Anela Kellogg, Mary McLaughlin, Maryellen McManus, Raul M Alfaro, Colleen Hadigan, Joseph A Kovacs, Parag Kumar
Background: Once-weekly isoniazid and rifapentine for 3 months is a treatment option in persons with human immunodeficiency virus and latent tuberculosis infection. This study aimed to examine pharmacokinetic drug-drug interactions between this regimen and dolutegravir, a first-line antiretroviral medication. Methods: This was a single-center, open-label, fixed-sequence, drug-drug interaction study in healthy volunteers. Subjects received oral dolutegravir 50 mg once-daily alone (Days 1-4) and concomitantly with once-weekly isoniazid 900 mg, rifapentine 900 mg, and pyridoxine 50 mg (Days 5-19)...
February 3, 2018: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
Oscar Pardo-Planas, Hasan K Atiyeh, Rolf A Prade, Michael Müller, Mark R Wilkins
An A. nidulans strain with a pyridoxine marker was used for continuous production of aryl alcohol oxidase (AAO) in a trickle bed reactor (TBR). Modified medium with reduced zinc, no copper, and 5 g/L ascorbic acid that reduced melanin production and increased AAO productivity under growth limited conditions was used. Two air flow rates, 0.11 L/min (0.1 vvm) and 1.1 L/min (1.0 vvm) were tested. More melanin formation and reduced protein productivity were observed with air flow rate of 1.1 L/min. Three random packings were used as support for the fungus inside the TBR column, two of which were hydrophobic and one which was hydrophilic, and three different dilution rates were tested...
February 2, 2018: Bioresource Technology
Shakeel Shahzad, M Adnan Ashraf, M Sajid, Aqeel Shahzad, Azhar Rafique, M Shahid Mahmood
BACKGROUND & OBJECTIVES: Multiple drug resistant super bugs of Acinetobacter baumannii and methicillin-resistant Staphylococcus aureus (MRSA) are becoming challenge for healthcare professionals. In this study, vitamins were evaluated for synergistic activity with the antibiotics. METHODS: Synergistic effect between antibiotic and stock solutions of vitamins is evaluated by using Kirby-Bauer disc diffusion assay. Distilled water and propylene glycol were used as solvent for water soluble vitamins and fat-soluble vitamins respectively...
February 2, 2018: Journal of Global Antimicrobial Resistance
C Fonta, J-P Salles
The pathophysiology of the neuromuscular manifestations of hypophosphatasia (HPP) remains unknown. Pyridoxine-sensitive seizures characterize severe forms of infantile HPP. Young children and infants affected with severe forms of HPP, but also adults often present with myopathy characterized by hypotonia or muscle weakness. Chronic pain, of unclear mechanism is also often present. Tissue-non-specific alkaline phosphatase (Alkaline Phosphatase-Liver/Bone/Kidney [ALPL]) is expressed in brain neuronal cell and in muscle cells during development and adulthood...
May 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Rangan Srinivasaraghavan, Narayanan Parameswaran, Deborah Mathis, Celine Bürer, Barbara Plecko
Antiquitin deficiency is the most prevalent form of pyridoxine-dependent epilepsy. While most patients present with neonatal onset of therapy-resistant seizures, a few cases with late-onset during infancy have been described. Here, we describe the juvenile onset of epilepsy at the age of 17 years due to antiquitin deficiency in an Indian female with homozygosity for the most prevalent ALDH7A1 missense mutation, c.1279G > C; p.Glu427Gln in exon 14. The diagnosis was established along familial cosegregation analysis for an affected offspring, that had neonatal pyridoxine responsive seizures and had been found to be compound heterozygous for c...
February 5, 2018: Neuropediatrics
Miguel Glatstein, Gary Carbell, Dennis Scolnik, Ayelet Rimon, Shireen Banerji, Christopher Hoyte
No abstract text is available yet for this article.
January 31, 2018: American Journal of Emergency Medicine
Hung Yi Song, Roch Chui Yu
γ-Aminobutyric acid (GABA), a nonprotein amino acid, is widely distributed in nature and fulfills several physiological functions. In this study, various lactic acid strains commonly used to produce fermented milk products were inoculated into adzuki bean milk for producing GABA. The high GABA producing strain was selected in further experiment to improve the GABA production utilizing culture medium optimization. The results demonstrated that adzuki bean milk inoculated with Lactobacillus rhamnosus GG increased GABA content from 0...
January 2018: Journal of Food and Drug Analysis
Roseli B D Toffano, Elaine Hillesheim, Mariana G Mathias, Carolina A Coelho-Landell, Roberta G Salomão, Maria O R V Almada, Joyce M Camarneiro, Tamiris T Barros, José S Camelo-Junior, Serge Rezzi, Laurence Goulet, Maria P Giner, Laeticia Da Silva, Francois-Pierre Martin, Ivan Montoliu, Sofia Moco, Sebastiano Collino, Jim Kaput, Jacqueline P Monteiro
The Brazilian Healthy Eating Index-Revised (BHEI-R) can be used to determine overall dietary patterns. We assessed the BHEI-R scores in children and adolescents, aged from 9 to 13 years old, and associated its component scores with biomarkers of health and dietary exposure. Three 24-h recalls were used to generate BHEI-R. Biomarkers were analyzed in plasma and red blood cells. Correlation tests, agreement, and covariance analyses were used to associate BHEI-R components with biomarkers. Data from 167 subjects were used...
January 30, 2018: Nutrients
Roman Maslanka, Magdalena Kwolek-Mirek, Renata Zadrag-Tecza
Autofluorescence is the natural fluorescence emitted by cellular compounds which have light emission properties. The main examples of these compounds, identified as an endogenous fluorophores, include aromatic amino acids, vitamins, coenzymes and electron acceptors. As many of them play a critical role in cell metabolism, changes in their content may provide important information on the physiological status of the cell. Nevertheless, the simultaneous occurrence of different endogenous fluorophores in cells makes it difficult to interpret the autofluorescence signal...
January 27, 2018: Journal of Microbiological Methods
Martino L di Salvo, Mario Mastrangelo, Isabel Nogués, Manuela Tolve, Alessandro Paiardini, Carla Carducci, Davide Mei, Martino Montomoli, Angela Tramonti, Renzo Guerrini, Roberto Contestabile, Vincenzo Leuzzi
PNPO deficiency is responsible of severe neonatal encephalopathy, responsive to pyridoxal-5'-phosphate (PLP) or pyridoxine. Recent studies widened the phenotype of this condition and detected new genetic variants on PNPO gene, whose pathogenetic role and clinical expression remain to be established. One of these mutations, Arg116Gln, is of particular interest because of its later onset of symptoms (beyond the first months of life) and its peculiar epileptic manifestations in patients. This protein variant was expressed as recombinant protein in E coli, purified to homogeneity, and characterized with respect to structural and kinetic properties, stability, binding constants of cofactor flavin mononucleotide (FMN) and product (PLP) in order to define the molecular and structural bases of its pathogenicity...
December 2017: Data in Brief
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