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https://www.readbyqxmd.com/read/28932990/treatment-opportunities-in-patients-with-metabolic-myopathies
#1
REVIEW
Mette Cathrine Ørngreen, John Vissing
Metabolic myopathies are disorders affecting utilization of carbohydrates or fat in the skeletal muscle. Adult patients with metabolic myopathies typically present with exercise-induced pain, contractures or stiffness, fatigue, and myoglobinuria. Symptoms are related to energy failure. Purpose of review In this review, the current treatment options, including exercise therapy, dietary treatment, pharmacological supplementation, gene transcription, and enzyme replacement therapy, are described. Recent findings Recognition of the metabolic block in the metabolic myopathies has started the development of new therapeutic options...
September 21, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28911109/selective-tumor-cell-death-induced-by-irradiated-riboflavin-through-recognizing-dna-g-t-mismatch
#2
Yi Yuan, Yongyun Zhao, Lianqi Chen, Jiasi Wu, Gangyi Chen, Sheng Li, Jiawei Zou, Rong Chen, Jian Wang, Fan Jiang, Zhuo Tang
Riboflavin (vitamin B2) has been thought to be a promising antitumoral agent in photodynamic therapy, though the further application of the method was limited by the unclear molecular mechanism. Our work reveals that riboflavin was able to recognize G-T mismatch specifically and induce single-strand breaks in duplex DNA targets efficiently under irradiation. In the presence of riboflavin, the photo-irradiation could induce the death of tumor cells that are defective in mismatch repair system selectively, highlighting the G-T mismatch as potential drug target for tumor cells...
September 6, 2017: Nucleic Acids Research
https://www.readbyqxmd.com/read/28821122/-click-hyaluronan-based-nanohydrogels-as-multifunctionalizable-carriers-for-hydrophobic-drugs
#3
Giuliana Manzi, Nicole Zoratto, Silvia Matano, Rocchina Sabia, Claudio Villani, Tommasina Coviello, Pietro Matricardi, Chiara Di Meo
Highly hydrophilic and biocompatible nanocarriers based on polysaccharide hydrogels (nanohydrogels, NHs) were shown to be promising systems for drug delivery applications. Following the idea of these emerging drug carriers, the aim of the present work was to develop self-assembled hydrogel nanoparticles based on amphiphilic derivatives of hyaluronic acid (HA) and riboflavin (Rfv), synthesized by "click" Copper(I)-catalyzed Azide-Alkyne Cycloaddition (CuAAC) reaction. The obtained amphiphilic product (HA-c-Rfv) was able to form nanohydrogels in aqueous environments, in particular by applying an innovative autoclave-based method...
October 15, 2017: Carbohydrate Polymers
https://www.readbyqxmd.com/read/28688841/how-mr1-presents-a-pathogen-metabolic-signature-to-mucosal-associated-invariant-t-mait-cells
#4
REVIEW
Hamish E G McWilliam, Jose A Villadangos
Mucosal-associated invariant T (MAIT) cells are innate-like lymphocytes restricted by the antigen (Ag)-presenting molecule MHC class I (MHC I)-related protein 1 (MR1). The Ags presented by MR1 are vitamin B-related Ags (VitBAgs), 'building-block' metabolites of riboflavin that are synthesized by a range of microbes. MR1 presentation is thus a unique mechanism for the immune detection of a pathogen metabolic signature. While the full picture of how MR1 accomplishes this remains incomplete, recent data show that, unlike other MHC molecules, MR1 operates by a presentation-on-demand mechanism...
July 5, 2017: Trends in Immunology
https://www.readbyqxmd.com/read/28665138/alternative-electron-transfer-channels-ensure-ultrafast-deactivation-of-light-induced-excited-states-in-riboflavin-binding-protein
#5
Laura Zanetti-Polzi, Massimiliano Aschi, Andrea Amadei, Isabella Daidone
Flavoproteins, containing flavin chromophores, are enzymes capable of transferring electrons at very high speeds. The ultrafast photoinduced electron-transfer (ET) kinetics of riboflavin binding protein to the excited riboflavin was studied by femtosecond spectroscopy and found to occur within a few hundred femtoseconds [ Zhong and Zewail, Proc. Natl. Acad. Sci. U.S.A. 2001, 98, 11867-11872 ]. This ultrafast kinetics was attributed to the presence of two aromatic rings that could transfer the electron to riboflavin: the side chains of tryptophan 156 and tyrosine 75...
July 7, 2017: Journal of Physical Chemistry Letters
https://www.readbyqxmd.com/read/28654805/role-of-root-exudates-in-metal-acquisition-and-tolerance
#6
REVIEW
Yi-Tze Chen, Ying Wang, Kuo-Chen Yeh
Plants acquire mineral nutrients mostly through the rhizosphere; they secrete a large number of metabolites into the rhizosphere to regulate nutrient availability and to detoxify undesirable metal pollutants in soils. The secreted metabolites are inorganic ions, gaseous molecules, and mainly carbon-based compounds. This review focuses on the mechanisms and regulation of low-molecular-weight organic-compound exudation in terms of metal acquisition. We summarize findings on riboflavin/phenolic-facilitated and phytosiderophore-facilitated iron acquisition and discuss recent studies of the functions and secretion mechanisms of low-molecular-weight organic acids in heavy-metal detoxification...
June 24, 2017: Current Opinion in Plant Biology
https://www.readbyqxmd.com/read/28637675/structure-functional-aspects-of-the-human-riboflavin-transporter-3-slc52a3-role-of-the-predicted-glycosylation-and-substrate-interacting-sites
#7
Veedamali S Subramanian, Subrata Sabui, Trevor Teafatiller, Jennifer A Bohl, Hamid M Said
The human riboflavin (RF) transporter-3 (hRFVT-3; product of the SLC52A3 gene) plays an essential role in the intestinal RF absorption process and is expressed exclusively at the apical membrane domain of polarized enterocytes. Previous studies have characterized different physiological/biological aspects of this transporter, but nothing is known about the glycosylation status of the hRFVT-3 protein and role of this modification in its physiology/biology. Additionally, little is known about the residues in the hRFVT-3 protein that interact with the ligand, RF...
August 1, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/28620369/characterization-of-bile-salt-hydrolase-from-lactobacillus-gasseri-fr4-and-demonstration-of-its-substrate-specificity-and-inhibitory-mechanism-using-molecular-docking-analysis
#8
Rizwana Parveen Rani, Marimuthu Anandharaj, Abraham David Ravindran
Probiotic bacteria are beneficial to the health of poultry animals, thus are used as alternative candidates for antibiotics used as growth promoters (AGPs). However, they also reduce the body weight gain due to innate bile salt hydrolase (BSH) activity. Hence, the addition of a suitable BSH inhibitor along with the probiotic feed can decrease the BSH activity. In this study, a BSH gene (981 bp) encoding 326-amino acids was identified from the genome of Lactobacillus gasseri FR4 (LgBSH). The LgBSH-encoding gene was cloned and purified using an Escherichia coli BL21 (DE3) expression system, and its molecular weight (37 kDa) was confirmed by SDS-PAGE and a Western blot analysis...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28572726/photoacoustic-imaging-of-tumor-targeting-with-riboflavin-functionalized-theranostic-nanocarriers
#9
Nataliia Beztsinna, Yoanna Tsvetkova, Jithin Jose, Boutayna Rhourri-Frih, Wa'el Al Rawashdeh, Twan Lammers, Fabian Kiessling, Isabelle Bestel
Photoacoustic imaging is an emerging method in the molecular imaging field, providing high spatiotemporal resolution and sufficient imaging depths for many clinical applications. Therefore, the aim of this study was to use photoacoustic imaging as a tool to evaluate a riboflavin (RF)-based targeted nanoplatform. RF is internalized by the cells through a specific pathway, and its derivatives were recently shown as promising tumor-targeting vectors for the drug delivery systems. Here, the RF amphiphile synthesized from a PEGylated phospholipid was successfully inserted into a long-circulating liposome formulation labeled with the clinically approved photoacoustic contrast agent - indocyanine green (ICG)...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28565913/outer-membrane-cytochromes-flavin-interactions-in-shewanella-spp-a-molecular-perspective
#10
Sofia Babanova, Ivana Matanovic, Jose Cornejo, Orianna Bretschger, Kenneth Nealson, Plamen Atanassov
Extracellular electron transfer (EET) is intrinsically associated with the core phenomena of energy harvesting/energy conversion in natural ecosystems and biotechnology applications. However, the mechanisms associated with EET are complex and involve molecular interactions that take place at the "bionano interface" where biotic/abiotic interactions are usually explored. This work provides molecular perspective on the electron transfer mechanism(s) employed by Shewanella oneidensis MR-1. Molecular docking simulations were used to explain the interfacial relationships between two outer-membrane cytochromes (OMC) OmcA and MtrC and riboflavin (RF) and flavin mononucleotide (FMN), respectively...
May 31, 2017: Biointerphases
https://www.readbyqxmd.com/read/28515315/the-c-terminal-peptide-of-aquifex-aeolicus-riboflavin-synthase-directs-encapsulation-of-native-and-foreign-guests-by-a-cage-forming-lumazine-synthase
#11
Yusuke Azuma, Reinhard Zschoche, Donald Hilvert
Encapsulation of specific enzymes in self-assembling protein cages is a hallmark of bacterial compartments that function as counterparts to eukaryotic organelles. The cage-forming enzyme lumazine synthase (LS) from Bacillus subtilis (BsLS), for example, encapsulates riboflavin synthase (BsRS), enabling channeling of lumazine from the site of its generation to the site of its conversion to vitamin B2 Elucidating the molecular mechanisms underlying the assembly of these supramolecular complexes could help inform new approaches for metabolic engineering, nanotechnology, and drug delivery...
June 23, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28475111/riboflavin-responsive-mitochondrial-dysfunction-in-neurodegenerative-diseases
#12
REVIEW
Tamilarasan Udhayabanu, Andreea Manole, Mohan Rajeshwari, Perumal Varalakshmi, Henry Houlden, Balasubramaniem Ashokkumar
Mitochondria are the repository for various metabolites involved in diverse energy-generating processes, like the TCA cycle, oxidative phosphorylation, and metabolism of amino acids, fatty acids, and nucleotides, which rely significantly on flavoenzymes, such as oxidases, reductases, and dehydrogenases. Flavoenzymes are functionally dependent on biologically active flavin adenine dinucleotide (FAD) or flavin mononucleotide (FMN), which are derived from the dietary component riboflavin, a water soluble vitamin...
May 5, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28456887/heterogeneous-phenotypes-in-lipid-storage-myopathy-due-to-etfdh-gene-mutations
#13
Corrado Angelini, Daniela Tavian, Sara Missaglia
We present six novel patients affected by lipid storage myopathy (LSM) presenting mutations in the ETFDH gene. Although the diagnosis of multiple acyl-coenzyme-A dehydrogenase deficiency (MADD) in adult life is difficult, it is rewarding because of the possibility of treating patients with carnitine or riboflavin, leading to a full recovery. In our patients, a combination of precipitating risk factors including previous anorexia, alcoholism, poor nutrition, and pregnancy contributed to a metabolic critical condition that precipitated the catabolic state...
April 30, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28411331/glutaric-acidemia-type-1-a-case-of-infantile-stroke
#14
Gül Demet Kaya Ozcora, Songul Gokay, Mehmet Canpolat, Fatih Kardaş, Mustafa Kendirci, Sefer Kumandaş
BACKGROUND: Glutaric acidemia Type 1 (GA-1) is an autosomal recessively inherited metabolic disorder which is associated with GCDH gene mutations which alters the glutaryl-CoA dehydrogenase, an enzyme playing role in the catabolic pathways of the amino acids lysine, hydroxylysine, and tryptophan. Clinical findings are often encephalopathic crises, dystonia, and extrapyramidal symptoms. CASE REPORT: A 9-month-old male infant referred to our department with focal tonic-clonic seizures during rotavirus infection and acute infarcts in MRI...
April 15, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28407967/molecular-characterization-of-an-unauthorized-genetically-modified-bacillus-subtilis-production-strain-identified-in-a-vitamin-b2-feed-additive
#15
Valentina Paracchini, Mauro Petrillo, Ralf Reiting, Alexandre Angers-Loustau, Daniela Wahler, Andrea Stolz, Birgit Schönig, Anastasia Matthies, Joachim Bendiek, Dominik M Meinel, Sven Pecoraro, Ulrich Busch, Alex Patak, Joachim Kreysa, Lutz Grohmann
Many food and feed additives result from fermentation of genetically modified (GM) microorganisms. For vitamin B2 (riboflavin), GM Bacillus subtilis production strains have been developed and are often used. The presence of neither the GM strain nor its recombinant DNA is allowed for fermentation products placed on the EU market as food or feed additive. A vitamin B2 product (80% feed grade) imported from China was analysed. Viable B. subtilis cells were identified and DNAs of two bacterial isolates (LHL and LGL) were subjected to three whole genome sequencing (WGS) runs with different devices (MiSeq, 454 or HiSeq system)...
September 1, 2017: Food Chemistry
https://www.readbyqxmd.com/read/28394446/laser-ablation-electrospray-ionization-mass-spectrometry-with-ion-mobility-separation-reveals-metabolites-in-the-symbiotic-interactions-of-soybean-roots-and-rhizobia
#16
Sylwia A Stopka, Beverly J Agtuca, David W Koppenaal, Ljiljana Paša-Tolić, Gary Stacey, Akos Vertes, Christopher R Anderton
Technologies enabling in situ metabolic profiling of living plant systems are invaluable for understanding physiological processes and could be used for rapid phenotypic screening (e.g., to produce plants with superior biological nitrogen-fixing ability). The symbiotic interaction between legumes and nitrogen-fixing soil bacteria results in a specialized plant organ (i.e., root nodule) where the exchange of nutrients between host and endosymbiont occurs. Laser-ablation electrospray ionization mass spectrometry (LAESI-MS) is a method that can be performed under ambient conditions requiring minimal sample preparation...
April 10, 2017: Plant Journal: for Cell and Molecular Biology
https://www.readbyqxmd.com/read/28367977/riboflavin-deficiency-induces-a-significant-change-in-proteomic-profiles-in-hepg2-cells
#17
Zhonghao Xin, Lingling Pu, Weina Gao, Yawen Wang, Jingyu Wei, Tala Shi, Zhanxin Yao, Changjiang Guo
Riboflavin deficiency is widespread in many regions over the world, especially in underdeveloped countries. In this study, we investigated the effects of riboflavin deficiency on protein expression profiles in HepG2 cells in order to provide molecular information for the abnormalities induced by riboflavin deficiency. HepG2 cells were cultured in media containing different concentrations of riboflavin. Changes of cell viability and apoptosis were assessed. A comparative proteomic analysis was performed using a label-free shotgun method with LC-MS/MS to investigate the global changes of proteomic profiles in response to riboflavin deficiency...
April 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28330106/enhanced-production-of-pectinase-by-aspergillus-terreus-ncft-4269-10-using-banana-peels-as-substrate
#18
Bijay Kumar Sethi, Prativa Kumari Nanda, Santilata Sahoo
Aspergillus terreus NCFT4269.10 was implemented in solid-state (SSF) and liquid static surface fermentation (LSSF) for biosynthesis of pectinase. Amongst various substrates, like, mustard oil cake, neem oil cake, groundnut oil cake, black gram peels, green gram peels, chickling vetch peels/grass pea peels wheat bran, pearl millet residues, finger millet waste, broken rice, banana peels (BP), apple pomace (AP) and orange peels, banana peel (Musa paradisiaca L.; Family: Musaceae) was most suitable for pectinase biosynthesis (LSSF: 400 ± 21...
June 2016: 3 Biotech
https://www.readbyqxmd.com/read/28325140/vitamins-in-heart-failure-friend-or-enemy
#19
George Georgiopoulos, Christina Chrysohoou, Georgia Vogiatzi, Nikolaos Magkas, Ippokratis Bournelis, Sofia Bampali, Damien Gruson, Dimitris Tousoulis
BACKGROUND: The failing heart is characterized by a depleted metabolic energy reserve and the up-regulation of several molecular mechanisms leading to cardiac hypertrophy, inflammation, fibrosis, angiogenesis, and apoptosis. Dietary or non-dietary supplementation of vitamins could potentially benefit energy balance. OBJECTIVE: The objective of the present study was to evaluate all available information on vitamins supplementation in patients with chronic HF for possible beneficial effect on metabolic, inotropic, chronotropic and hemodynamic indices...
March 20, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28276274/multi-spectroscopic-and-molecular-modelling-approach-to-investigate-the-interaction-of-riboflavin-with-human-serum-albumin
#20
Md Maroof Alam, Faizan Abul Qais, Iqbal Ahmad, Parvez Alam, Rizwan Hasan Khan, Imrana Naseem
Riboflavin (RF) plays an important role in various metabolic redox reactions in the form of flavin adenine dinucleotide and flavin mononucleotide. Human serum albumin (HSA) is an important protein involved in the transportation of drugs, hormones, fatty acid and other molecules which determine the biodistribution and physiological fate of these molecules. In this study, we have investigated the interaction of riboflavin RF with HSA under simulative physiological conditions using various biophysical, calorimetric and molecular docking techniques...
March 9, 2017: Journal of Biomolecular Structure & Dynamics
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