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https://www.readbyqxmd.com/read/27932481/modeling-monogenic-human-nephrotic-syndrome-in-the-drosophila-garland-cell-nephrocyte
#1
Tobias Hermle, Daniela A Braun, Martin Helmstädter, Tobias B Huber, Friedhelm Hildebrandt
Steroid-resistant nephrotic syndrome is characterized by podocyte dysfunction. Drosophila garland cell nephrocytes are podocyte-like cells and thus provide a potential in vivo model in which to study the pathogenesis of nephrotic syndrome. However, relevant pathomechanisms of nephrotic syndrome have not been studied in nephrocytes. Here, we discovered that two Drosophila slit diaphragm proteins, orthologs of the human genes encoding nephrin and nephrin-like protein 1, colocalize within a fingerprint-like staining pattern that correlates with ultrastructural morphology...
December 8, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27931034/historical-perspective-on-clinical-trials-of-carnitine-in-children-and-adults
#2
Neil R M Buist
The metabolic roles of carnitine have been greatly clarified over the past 50 years, and it is now well established that carnitine is a key player in mitochondrial generation of energy and metabolism of acetyl coenzyme A. A therapeutic role for carnitine in treatment of nutritional deficiencies in infants and children was first demonstrated in 1958, and since that time it has been used to treat a number of inborn errors of metabolism. Carnitine was approved by the US Food and Drug Administration in 1985 for treatment of 'primary carnitine deficiency', and later in 1992 for treatment of 'secondary carnitine deficiency', a definition that included the majority of relevant metabolic disorders associated with low or abnormal plasma carnitine levels...
December 9, 2016: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/27931031/round-table-discussion
#3
Susan Winter, Neil R M Buist, Nicola Longo, Saro H Armenian, Gary Lopaschuk, Anna Wasilewska
The 1st International Carnitine Working Group concluded with a round table discussion addressing several areas of relevance. These included the design of future studies that could increase the amount of evidence-based data about the role of carnitine in the treatment of fatty acid oxidation defects, for which substantial controversy still exists. There was general consensus that future trials on the effect of carnitine in disorders of fatty acid oxidation should be randomized, double-blinded, multicentered and minimally include the following diagnoses: medium-chain acyl coenzyme A (CoA) dehydrogenase deficiency, very long-chain acyl-CoA dehydrogenase deficiency, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and mitochondrial trifunctional protein deficiency...
December 9, 2016: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/27927588/effect-of-coenzyme-q10-on-biomarkers-of-oxidative-stress-and%C3%A2-cardiac-function-in-hemodialysis-patients-the-coq10-biomarker-trial
#4
Matthew B Rivara, Catherine K Yeung, Cassianne Robinson-Cohen, Brian R Phillips, John Ruzinski, Denise Rock, Lori Linke, Danny D Shen, T Alp Ikizler, Jonathan Himmelfarb
BACKGROUND: Oxidative stress is highly prevalent in patients with end-stage renal disease and is linked to excess cardiovascular risk. Identifying therapies that reduce oxidative stress has the potential to improve cardiovascular outcomes in patients undergoing maintenance dialysis. STUDY DESIGN: Placebo-controlled, 3-arm, double-blind, randomized, clinical trial. SETTING & PARTICIPANTS: 65 patients undergoing thrice-weekly maintenance hemodialysis...
December 4, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27924150/increasing-n-butanol-production-with-saccharomyces-cerevisiae-by-optimizing-acetyl-coa-synthesis-nadh-levels-and-trans-2-enoyl-coa-reductase-expression
#5
Virginia Schadeweg, Eckhard Boles
BACKGROUND: n-Butanol can serve as an excellent gasoline substitute. Naturally, it is produced by some Clostridia species which, however, exhibit only limited suitability for industrial n-butanol production. The yeast Saccharomyces cerevisiae would be an ideal host due to its high robustness in fermentation processes. Nevertheless, n-butanol yields and titers obtained so far with genetically engineered yeast strains are only low. RESULTS: In our recent work, we showed that n-butanol production via a clostridial acetoacetyl-CoA-derived pathway in engineered yeast was limited by the availability of coenzyme A (CoA) and cytosolic acetyl-CoA...
2016: Biotechnology for Biofuels
https://www.readbyqxmd.com/read/27920436/a-statistical-algorithm-showing-coenzyme-q10-and-citrate-synthase-as-biomarkers-for-mitochondrial-respiratory-chain-enzyme-activities
#6
D Yubero, A Adin, R Montero, C Jou, C Jiménez-Mallebrera, A García-Cazorla, A Nascimento, M M O'Callaghan, J Montoya, L Gort, P Navas, A Ribes, M D Ugarte, R Artuch
Laboratory data interpretation for the assessment of complex biological systems remains a great challenge, as occurs in mitochondrial function research studies. The classical biochemical data interpretation of patients versus reference values may be insufficient, and in fact the current classifications of mitochondrial patients are still done on basis of probability criteria. We have developed and applied a mathematic agglomerative algorithm to search for correlations among the different biochemical variables of the mitochondrial respiratory chain in order to identify populations displaying correlation coefficients >0...
December 2016: Scientific Reports
https://www.readbyqxmd.com/read/27920298/malate-synthase-and-%C3%AE-methylmalyl-coa-lyase-reactions-in-the-methylaspartate-cycle-in-haloarcula-hispanica
#7
Farshad Borjian, Jing Han, Jing Hou, Hua Xiang, Jan Zarzycki, Ivan A Berg
: Haloarchaea are extremely halophilic heterotrophic microorganisms belonging to the class Halobacteria (Euryarchaeota). Almost half of the haloarchaea possesses the genes coding for enzymes of the methylaspartate cycle, a recently discovered anaplerotic acetate assimilation pathway. In this cycle, the enzymes of the tricarboxylic acid cycle together with the dedicated enzymes of the methylaspartate cycle convert two acetyl-CoA molecules to malate. The methylaspartate cycle involves two reactions catalyzed by homologous enzymes belonging to the CitE-like enzyme superfamily, the malyl-CoA lyase/thioesterase (haloarchaeal malate synthase, hMS, Hah_2476 in Haloarcula hispanica) and the β-methylmalyl-CoA lyase (hMCL, Hah_1341)...
December 5, 2016: Journal of Bacteriology
https://www.readbyqxmd.com/read/27920295/a-novel-transcriptional-regulator-related-to-thiamine-phosphate-synthase-controls-thiamine-metabolism-genes-in-archaea
#8
Dmitry A Rodionov, Semen A Leyn, Xiaoqing Li, Irina A Rodionova
: Thiamine (vitamin B1) is a precursor of thiamine pyrophosphate (TPP), an essential coenzyme in the central metabolism of all living organisms. Bacterial thiamin biosynthesis and salvage genes are controlled at the RNA level by TPP-responsive riboswitches. In Archaea, TPP riboswitches are restricted to the Thermoplasmatales order. Mechanisms of transcriptional control of thiamine genes in other archaeal lineages remain unknown. Using the comparative genomics approach, we identified a novel family of transcriptional regulators (named ThiR) controlling thiamine biosynthesis and transport genes in diverse lineages from the Crenarchaeota phylum, as well as in the Halobacteria and Thermococci classes of the Euryarchaeota ThiR regulators are composed of an N-terminal DNA-binding domain and a C-terminal ligand-binding domain that is similar to the archaeal thiamin phosphate synthase ThiN...
December 5, 2016: Journal of Bacteriology
https://www.readbyqxmd.com/read/27919791/biosensor-assisted-transcriptional-regulator-engineering-for-methylobacterium-extorquens-am1-to-improve-mevalonate-synthesis-by-increasing-the-acetyl-coa-supply
#9
Wei-Fan Liang, Lan-Yu Cui, Jin-Yu Cui, Kai-Wen Yu, Song Yang, Chong Zhang, Xin-Hui Xing
Acetyl-CoA is not only an important intermediate metabolite for cells but also a significant precursor for production of industrially interesting metabolites. Methylobacterium extorquens AM1, a model strain of methylotrophic cell factories using methanol as carbon source, is of interest because it produces abundant coenzyme A compounds capable of directing to synthesis of different useful compounds from methanol. However, acetyl-CoA always does not efficiently accumulate in M. extorquens AM1, as it is located in the center of three cyclic central metabolic pathways...
December 3, 2016: Metabolic Engineering
https://www.readbyqxmd.com/read/27917506/comparative-in-vitro-toxicity-assessment-of-perfluorinated-carboxylic-acids
#10
Cecon T Mahapatra, Nur P Damayanti, Samuel C Guffey, Jennifer S Serafin, Joseph Irudayaraj, Maria S Sepúlveda
Perfluoroalkyl and polyfluoroalkyl substances (PFASs) are synthetic fluorinated compounds that are highly bioaccumulative and persistent organic pollutants. Perfluorooctanoic acid (PFOA), an eight-carbon chain perfluorinated carboxylic acid, was used heavily for the production of fluoropolymers, but concerns have led to its replacement by shorter carbon chain homologues such as perfluorohexanoic acid (PFHxA) and perfluorobutanoic acid (PFBA). However, limited toxicity data exist for these substitutes. We evaluated the toxicity of PFOA, PFHxA and PFBA on a zebrafish liver cell line and investigated the effects of exposure on cell metabolism...
December 5, 2016: Journal of Applied Toxicology: JAT
https://www.readbyqxmd.com/read/27917474/germacrone-inhibits-adipogenesis-and-stimulates-lipolysis-via-the-amp-activated-protein-kinase-signalling-pathway-in-3t3-l1-preadipocytes
#11
Yuan-Ri Guo, Se-Young Choung
OBJECTIVES: In a previous study, we reported that Aster spathulifolius Maxim extract (ASE) inhibited lipid accumulation and adipocyte differentiation in 3T3-L1 cells. Of the components in ASE, germacrone (GM) was identified as a potent bioactive constituent. GM is known for its anticancer and antiviral activity. However, the effects of GM and the molecular mechanism by which GM regulates adipogenesis and lipolysis were not reported. Therefore, we investigated the effect of GM on adipogenesis and lipolysis and to elucidate its underlying molecular mechanism...
December 5, 2016: Journal of Pharmacy and Pharmacology
https://www.readbyqxmd.com/read/27916754/-autoantibodies-of-inflammatory-myopathies-update
#12
Shigeaki Suzuki
Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve the skeletal muscle as well as many other organs. In addition to a histological diagnosis at muscle biopsy, the clinical phenotypes of inflammatory myopathies can be defined by the presence of various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, the correlation between histological features and autoantibodies has not been fully elucidated. Immune-mediated necrotizing myopathy (IMNM), which is characterized by significant necrotic and regeneration muscle fibers with minimal or no inflammatory cell infiltration, is associated with the presence of autoantibodies...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27916753/-diagnosis-of-idiopathic-inflammatory-myopathy-a-muscle-pathology-perspective
#13
Michio Inoue, Ichizo Nishino
Idiopathic inflammatory myopathies are historically classified into polymyositis and dermatomyositis based on the presence or absence of skin lesions. Recently, however, a more histology-oriented classification into 6 subtypes has been proposed. The subtypes include dermatomyositis, polymyositis, inclusion body myositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome, and non-specific myositis. With strict criteria applied, polymyositis is now extremely rare, while immune-mediated necrotizing myopathy is the most common among all inflammatory myopathies and is often associated with autoantibodies including those for signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR)...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27914961/expanding-the-clinical-and-genetic-spectrum-of-g6pd-deficiency-the-occurrence-of-bcgitis-and-novel-missense-mutation
#14
Taj Ali Khan, Humaira Mazhar, Mehboob Nawaz, Kalsoom Kalsoom, Muhammad Ishfaq, Huma Asif, Hazir Rahman, Muhammad Qasim, Farkhanda Naz, Mubashir Hussain, Baharullah Khattak, Waheed Ullah, Otavio Cabral-Marques, Jawad Butt, Asif Iqbal
Glucose-6-phosphate dehydrogenase (G6PD) is a key enzyme in the pentose phosphate pathway that ensures sufficient production of coenzyme nicotinamide adenine dinucleotide phosphate (NADPH) by catalyzing the reduction of NADP+ to NADPH. Noteworthy, the latter mediates the production of reactive oxygen species (ROS) by phagocytic cells such as neutrophils and monocytes. Therefore, patients with severe forms of G6PD deficiency may present impaired NADPH oxidase activity and become susceptible to recurrent infections...
November 30, 2016: Microbial Pathogenesis
https://www.readbyqxmd.com/read/27913825/hypolipidemic-effect-of-%C3%AE-caryophyllene-to-treat-hyperlipidemic-rats
#15
Matheus D Baldissera, Carine F Souza, Thirssa H Grando, Pedro H Doleski, Aline A Boligon, Lenita M Stefani, Silvia G Monteiro
The aim of this study was to evaluate the effect of β-caryophyllene on hypercholesterolemia using a model of hyperlipidemia induced by Triton WR-1339 in rats, as well as its possible effect on hepatic antioxidant enzymes. Thus, total cholesterol, triglycerides, low-density lipoprotein (LDL) cholesterol, and high-density lipoprotein (HDL) cholesterol were measured in serum, while reactive oxygen species (ROS), thiobarbituric acid reactive substances (TBARS), hepatic 3-hydroxy-3-methylglutayl coenzyme A (HMG-CoA) reductase, superoxide dismutase (SOD), and catalase (CAT) activities were measured in the hepatic tissue...
December 2, 2016: Naunyn-Schmiedeberg's Archives of Pharmacology
https://www.readbyqxmd.com/read/27913695/a-randomized-double-blind-placebo-controlled-trial-of-coenzyme-q10-in-huntington-disease
#16
Andrew McGarry, Michael McDermott, Karl Kieburtz, Elisabeth A de Blieck, Flint Beal, Karen Marder, Christopher Ross, Ira Shoulson, Peter Gilbert, William M Mallonee, Mark Guttman, Joanne Wojcieszek, Rajeev Kumar, Mark S LeDoux, Mary Jenkins, H Diana Rosas, Martha Nance, Kevin Biglan, Peter Como, Richard M Dubinsky, Kathleen M Shannon, Padraig O'Suilleabhain, Kelvin Chou, Francis Walker, Wayne Martin, Vicki L Wheelock, Elizabeth McCusker, Joseph Jankovic, Carlos Singer, Juan Sanchez-Ramos, Burton Scott, Oksana Suchowersky, Stewart A Factor, Donald S Higgins, Eric Molho, Fredy Revilla, John N Caviness, Joseph H Friedman, Joel S Perlmutter, Andrew Feigin, Karen Anderson, Ramon Rodriguez, Nikolaus R McFarland, Russell L Margolis, Eric S Farbman, Lynn A Raymond, Valerie Suski, Sandra Kostyk, Amy Colcher, Lauren Seeberger, Eric Epping, Sherali Esmail, Nancy Diaz, Wai Lun Alan Fung, Alan Diamond, Samuel Frank, Philip Hanna, Neal Hermanowicz, Leon S Dure, Merit Cudkowicz
OBJECTIVE: To test the hypothesis that chronic treatment of early-stage Huntington disease (HD) with high-dose coenzyme Q10 (CoQ) will slow the progressive functional decline of HD. METHODS: We performed a multicenter randomized, double-blind, placebo-controlled trial. Patients with early-stage HD (n = 609) were enrolled at 48 sites in the United States, Canada, and Australia from 2008 to 2012. Patients were randomized to receive either CoQ 2,400 mg/d or matching placebo, then followed for 60 months...
December 2, 2016: Neurology
https://www.readbyqxmd.com/read/27912736/citrus-junos-tanaka-peel-ameliorates-hepatic-lipid-accumulation-in-hepg2-cells-and-in-mice-fed-a-high-cholesterol-diet
#17
Eun Ju Shin, Jae Ho Park, Mi Jeong Sung, Min-Yu Chung, Jin-Taek Hwang
BACKGROUND: Citrus junos Tanaka (yuja), a yellow-coloured citrus fruit has traditionally been consumed in Korea, Japan, and China and has been found effective in preventing certain diseases. However, the inhibitory effect of yuja on hepatic lipid accumulation has not been clearly elucidated thus far. METHODS: The inhibitory effect of yuja on hepatic lipid accumulation was investigated in both cell culture and mouse models. We investigated the inhibitory effect of ethanol extract of yuja peel (YE) using HepG2 cells...
December 3, 2016: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/27911471/the-effects-of-coenzyme-q10-supplementation-on-glucose-metabolism-and-lipid-profiles-in-women-with-polycystic-ovary-syndrome-a-randomized-double-blind-placebo-controlled-trial
#18
Mansooreh Samimi, Maryam Zarezade Mehrizi, Fatemeh Foroozanfard, Hossein Akbari, Mehri Jamilian, Shahnaz Ahmadi, Zatollah Asemi
BACKGROUND: Data on the effects of coenzyme Q10 (CoQ10) supplementation on metabolic profiles among subjects with polycystic ovary syndrome (PCOS) are scarce. OBJECTIVE: This study was done to evaluate the effects of CoQ10 supplementation on glucose metabolism and lipid profiles in subjects with PCOS. DESIGN, PATIENTS AND MEASUREMENTS: This randomized double-blind, placebo-controlled trial was conducted on 60 women diagnosed with PCOS. Subjects were randomly assigned into two groups to intake either 100 mg CoQ10 supplements (N=30) or placebo (N=30) per day for 12 weeks...
December 2, 2016: Clinical Endocrinology
https://www.readbyqxmd.com/read/27910744/peroxisome-proliferator-activated-receptor-alpha-ppar%C3%AE-a-key-regulator-of-lipid-metabolism-in-avians
#19
Bahman Navidshad, M Royan
Dietary fatty acids have various effects on cellular metabolism, and many of these effects are carried out through the alteration of the gene expression. Fatty acids upregulate or downregulate the expression of different genes by acting both as agonists or antagonists for nuclear hormone receptors. Peroxisome proliferator-activated receptors (PPARs) are members of the nuclear hormone receptor superfamily. To date, three PPAR subtypes, α, β, and γ, have been recognized. PPARs regulate various target genes with a role in intracellular and extracellular lipid metabolism, mainly those involved in peroxisomal β-oxidation...
2016: Critical Reviews in Eukaryotic Gene Expression
https://www.readbyqxmd.com/read/27909452/the-levels-of-cortisol-and-oxidative-stress-and-dna-damage-in-child-and-adolescent-victims-of-sexual-abuse-with-or-without-post-traumatic-stress-disorder
#20
Şeref Şimşek, Tuğba Yüksel, İbrahim Kaplan, Cem Uysal, Hüseyin Aktaş
OBJECTIVE: The aim of this study was to investigate whether cortisol and oxidative stress levels and DNA damage differ between individuals who developed PTSD or not following a sexual trauma. METHODS: The study included 61 children aged between 5 and 17 years who sustained sexual abuse (M/F: 18/43). The patients were divided into two groups: patients with PTSD and patients without PTSD based, based on the results of a structured psychiatric interview (K-SADS-PL and CAPS-CA)...
November 2016: Psychiatry Investigation
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