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https://www.readbyqxmd.com/read/29332587/lysosomal-acid-lipase-deficiency-could-dyslipidemia-drive-the-diagnosis
#1
Ornella Guardamagna
LAL-deficiency (LAL-D) is a rare and systemic condition, secondary to LIPA gene mutations, responsible for lysosomal accumulation of cholesteryl esters and triglycerides, whose manifestations are very heterogeneous in terms of age of onset, severity and type of clinical and radiological manifestations. Dyslipidemia, hepatomegaly and hepatosteatosis with increased levels of transaminases are the most common features. The increased risk of premature atherosclerosis and cardiovascular disorders,, secondary to a generalized alteration of lipid profile and lipoprotein dysfunction associated with LAL-D, has been increasingly pointed out...
January 11, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29332142/purification-and-characterization-of-a-novel-organic-solvent-tolerant-and-cold-adapted-lipase-from-psychrobacter-sp-zy124
#2
Yue Zhang, Fangling Ji, Jingyun Wang, Zhongji Pu, Bo Jiang, Yongming Bao
By screening 25 different psychrophilic strains isolated from the Arctic habitat, we isolated a strain capable of producing lipase. We identified this strain as Psychrobacter sp. ZY124 based on the amplified 16S rDNA sequence. The lipase, named as Lipase ZC12, produced from the supernatant of Psychrobacter sp. ZY124 cultured at 15 °C was purified to homogeneity by ammonium sulfate precipitation followed by Phenyl Sepharose FF gel hydrophobic chromatography. Based on the obtained amino acid sequence, Lipase ZC12 is classified as a member of the Proteus/psychrophilic subfamily of lipase family I...
January 13, 2018: Extremophiles: Life Under Extreme Conditions
https://www.readbyqxmd.com/read/29331635/sotatercept-with-long-term-extension-for-the-treatment-of-anaemia-in-patients-with-lower-risk-myelodysplastic-syndromes-a-phase-2-dose-ranging-trial
#3
Rami Komrokji, Guillermo Garcia-Manero, Lionel Ades, Thomas Prebet, David P Steensma, Joseph G Jurcic, Mikkael A Sekeres, Jesus Berdeja, Michael R Savona, Odile Beyne-Rauzy, Aspasia Stamatoullas, Amy E DeZern, Jacques Delaunay, Gautam Borthakur, Robert Rifkin, Thomas E Boyd, Abderrhamane Laadem, Bond Vo, Jennie Zhang, Marie Puccio-Pick, Kenneth M Attie, Pierre Fenaux, Alan F List
BACKGROUND: Myelodysplastic syndromes are characterised by ineffective erythropoiesis leading to anaemia. Sotatercept (ACE-011) is a novel activin receptor type IIA fusion protein that acts as a ligand trap to neutralise negative regulators of late-stage erythropoiesis. The aim of the study was to establish a safe and effective dose of sotatercept for the treatment of anaemia in patients with lower-risk myelodysplastic syndromes. METHODS: This open-label, multicentre, dose-ranging, phase 2 trial took place at 11 treatment centres in the USA and France...
January 10, 2018: Lancet Haematology
https://www.readbyqxmd.com/read/29331348/effects-of-l-tryptophan-on-the-growth-intestinal-enzyme-activities-and-non-specific-immune-response-of-sea-cucumber-apostichopus-japonicus-selenka-exposed-to-crowding-stress
#4
Endong Zhang, Shuanglin Dong, Fang Wang, Xiangli Tian, Qinfeng Gao
In order to reveal the effects of l-tryptophan (Trp) on the physiology and immune response of sea cucumber (Apostichopus japonicus Selenka) exposed to crowding stress, four density groups of sea cucumbers (i.e. 4, 8, 16 and 32 individuals per 40 L water, represented as L, ML, MH and H) were fed with diets containing 0, 1, 3 and 5% l-tryptophan respectively for 75 days. The results showed that the specific growth rates (SGR) of the sea cucumber fed with diet with 3% Trp (L, 2.1; ML, 1.76; MH, 1.2; H, 0.7) were significantly higher than those fed with basal diet without Trp supplementation (P < ...
January 10, 2018: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29331050/exercise-counteracts-lipotoxicity-by-improving-lipid-turnover-and-lipid-droplet-quality
#5
Evelyn Zacharewicz, Matthijs K C Hesselink, Patrick Schrauwen
The incidence of obesity and metabolic disease, such as type 2 diabetes mellitus (T2D), are rising globally. Dietary lipid over-supply leads to lipid accumulation at ectopic sites, such as skeletal muscle. Ectopic lipid storage is highly correlated with insulin resistance and T2D, likely due to a loss of metabolic flexibility - the capacity to switch between fat and glucose oxidation upon insulin stimulation - and cellular dysfunction because of lipotoxicity. However, muscle lipid levels are also elevated in endurance trained athletes, presenting a paradoxical phenotype of increased intramuscular lipids along with high insulin sensitivity - the "athletes' paradox"...
January 13, 2018: Journal of Internal Medicine
https://www.readbyqxmd.com/read/29330648/cloning-expression-and-characterization-of-the-esterase-estut1-from-ureibacillus-thermosphaericus-which-belongs-to-a-new-lipase-family-xviii
#6
Yuliya V Samoylova, Ksenia N Sorokina, Margarita V Romanenko, Valentin N Parmon
A new esterase gene from thermophilic bacteria Ureibacillus thermosphaericus was cloned into the pET32b vector and expressed in Escherichia coli BL21(DE3). Alignment of the estUT1 amino acid sequence revealed the presence of a novel canonical pentapeptide (GVSLG) and 41-47% identity to the closest family of the bacterial lipases XIII. Thus the esterase estUT1 from U. thermosphaericus was assigned as a member of the novel family XVIII. It also showed a strong activity toward short-chain esters (C2-C8), with the highest activity for C2...
January 12, 2018: Extremophiles: Life Under Extreme Conditions
https://www.readbyqxmd.com/read/29329853/ph-controlled-fermentation-in-mild-alkaline-conditions-enhances-bioactive-compounds-and-functional-features-of-lentil-to-ameliorate-metabolic-disturbances
#7
Sara Bautista-Expósito, Elena Peñas, José Manuel Silván, Juana Frias, Cristina Martínez-Villaluenga
Lentil fermentation has a promising potential as a strategy for development of multifunctional ingredients targeting metabolic syndrome (MetS). Response surface methodology was applied to optimize lentil fermentation and study its effects on generation of peptides, soluble phenolics and bioactivities. Fermentation using Lactobacillus plantarum and Savinase® 16 L was carried out at different pH (6.5-8.5) and times (5.5-30 h). Analysis of variance was performed to evaluate linear, quadratic and interaction effects between fermentation parameters...
May 15, 2018: Food Chemistry
https://www.readbyqxmd.com/read/29329320/atypical-e2f-functions-are-critical-for-pancreas-polyploidization
#8
Ramadhan B Matondo, Eva Moreno, Mathilda J M Toussaint, Peter C J Tooten, Saskia C van Essen, Elsbeth A van Liere, Sameh A Youssef, Laura Bongiovanni, Alain de Bruin
The presence of polyploid cells in the endocrine and exocrine pancreas has been reported for four decades. In rodents, pancreatic polyploidization is initiated after weaning and the number of polyploid cells increases with age. Surprisingly the molecular regulators and biological functions of polyploidization in the pancreas are still unknown. We discovered that atypical E2f activity is essential for polyploidization in the pancreas, using an inducible Cre/LoxP approach in new-born mice to delete ubiquitously the atypical E2f transcription factors, E2f7 and E2f8...
2018: PloS One
https://www.readbyqxmd.com/read/29327229/leopoldia-comosa-prevents-metabolic-disorders-in-rats-with-high-fat-diet-induced-obesity
#9
T Casacchia, F Scavello, C Rocca, M C Granieri, G Beretta, D Amelio, F Gelmini, A Spena, R Mazza, C C Toma, Tommaso Angelone, Giancarlo Statti, T Pasqua
PURPOSE: Obesity is the main feature of a complex illness known as metabolic syndrome. Anti-obesogenic therapies are often associated with side effects and represent a high cost in conventional pharmacological approaches. New strategies based on natural remedies are under continuous investigation. Leopoldia comosa (L.) Parl. (L. comosa) is a spontaneous plant with diuretic, anti-inflammatory and antioxidant properties. Recently, a hypoglycemic activity mediated by inhibition of carbohydrate digestion has been identified...
January 11, 2018: European Journal of Nutrition
https://www.readbyqxmd.com/read/29326160/hypoxia-inducible-lipid-droplet-associated-protein-inhibits-adipose-triglyceride-lipase
#10
Krishna M Padmanabha Das, Lisa Wechselberger, Márton Liziczai, Montserrat De la Rosa Rodriguez, Gernot F Grabner, Christoph Heier, Roland Viertlmayr, Claudia Radler, Joerg Lichtenegger, Robert Zimmermann, Jan Willem Borst, Rudolf Zechner, Sander Kersten, Monika Oberer
Elaborate control mechanisms of intracellular triacylglycerol (TAG) breakdown are critically involved in the maintenance of energy homeostasis. Hypoxia inducible lipid droplet associated protein (HILPDA)/Hypoxia inducible gene-2 (Hig-2) has been shown to affect intracellular TAG levels, yet, the underlying molecular mechanisms are unclear. Here, we show that HILPDA inhibits adipose triglyceride lipase (ATGL), the enzyme catalyzing the first step of intracellular TAG hydrolysis. HILPDA shares structural similarity with G0/G1 switch gene 2 (G0S2), an established inhibitor of ATGL...
January 11, 2018: Journal of Lipid Research
https://www.readbyqxmd.com/read/29324678/immobilization-of-enzymes-on-a-phospholipid-bionically-modified-polysulfone-gradient-pore-membrane-for-the-enhanced-performance-of-enzymatic-membrane-bioreactors
#11
Yizong Guo, Xueyan Zhu, Fei Fang, Xiao Hong, Huimin Wu, Dajing Chen, Xiaojun Huang
Enzymatic membrane bioreactors (EMBRs), with synergistic catalysis-separation performance, have increasingly been used for practical applications. Generally, the membrane properties, particularly the pore structures and interface interactions, have a significant impact on the catalytic efficiency of the EMBR. Therefore, a biomimetic interface based on a phospholipid assembled onto a polysulfone hollow-fiber membrane with perfect radial gradient pores (RGM-PSF) has been prepared in this work to construct a highly efficient and stable EMBR...
January 11, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29323738/an-epididymis-specific-secretory-protein-clpsl2-critically-regulates-sperm-motility-acrosomal-integrity-and-male-fertility
#12
Xiaosheng Lu, Fei Ding, Zizhen Lian, Lei Chen, Zuowu Cao, Yiqing Guan, Ran Chen, Dongqing Cai, Yanhong Yu
The epididymis performs an important role in the maturation of spermatozoa including their acquisition of progressive motility and fertilizing ability. However, the molecular mechanisms that govern these maturational events are still poorly defined. Here we report that Clpsl2, a novel colipase homology, is exclusively expressed in the caupt epididymis and conserved in mammalian. Clpsl2 was secreted into the lumen and covered the acrosome region and principal piece of spermatozoa tail. And during epididymal transit, the binding rate between Clpsl2 protein and the spermatozoa gradually decreased...
January 11, 2018: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/29322108/production-of-biodiesel-by-enzymatic-transesterification-of-waste-sardine-oil-and-evaluation-of-its-engine-performance
#13
A Arumugam, V Ponnusami
Waste sardine oil, a byproduct of fish industry, was employed as a low cost feedstock for biodiesel production. It has relatively high free fatty acid (FFA) content (32 mg KOH/g of oil). Lipase enzyme immobilized on activated carbon was used as the catalyst for the transesterification reaction. Process variables viz. reaction temperature, water content and oil to methanol molar ratio were optimized. Optimum methanol to oil molar ratio, water content and temperature were found to be 9:1, 10 v/v% and 30 °C respectively...
December 2017: Heliyon
https://www.readbyqxmd.com/read/29322001/evaluation-of-the-antidiabetic-property-of-aqueous-leaves-extract-of%C3%A2-zanthoxylum-armatum-dc-using-in%C3%A2-vivo-and-in%C3%A2-vitro-approaches
#14
Carey Vana Rynjah, Ningthoujam Neelima Devi, Nasheman Khongthaw, Donkupar Syiem, Suktilang Majaw
The present study was designed to evaluate the antidiabetic potential of the aqueous leaves extract of Zanthoxylum armatum DC. leaves using in vivo and in vitro approaches. For in vivo studies, blood glucose level was monitored at different intervals after administration of varying doses of the extract for its hypoglycemic (100-6000 mg/kg b.w.) and antihyperglycemic (250 mg/kg b.w.) effect in normoglycemic and diabetic mice. In vitro enzymatic inhibition activity was tested against α-amylase, α- and β-glucosidase and lipase...
January 2018: Journal of Traditional and Complementary Medicine
https://www.readbyqxmd.com/read/29320799/new-phenylethanoid-glycosides-from-cistanche-phelypaea-and-their-activity-as-inhibitors-of-monoacylglycerol-lipase-magl
#15
Khadidja Aya Beladjila, Djemaa Berrehal, Nunziatina De Tommasi, Carlotta Granchi, Giulia Bononi, Alessandra Braca, Marinella De Leo
Four new phenylethanoid glycosides (1: -4: ), 1-β-p-hydroxyphenyl-ethyl-2-O-acetyl-3,6-di-α-l-rhamnopyranosyl-β-d-glucopyranoside (1: ), 1-β-p-hydroxyphenyl-ethyl-3,6-O-di-α-l-rhamnopyranosyl-β-d-glucopyranoside (2: ), 1-β-p-hydroxyphenyl-ethyl-2-O-acetyl-3,6-di-α-l-rhamnopyranosyl-4-p-coumaroyl-β-d-glucopyranoside (3: ), and 1-β-p-hydroxyphenyl-ethyl-3,6-di-α-l-rhamnopyranosyl-4-p-coumaroyl-β-d-glucopyranoside (4: ), together with three known compounds, were isolated from the n-butanol extract of Cistanche phelypaea aerial parts...
January 10, 2018: Planta Medica
https://www.readbyqxmd.com/read/29320510/adipose-tissue-atgl-modifies-the-cardiac-lipidome-in-pressure-overload-induced-left-ventricular-failure
#16
Janek Salatzki, Anna Foryst-Ludwig, Kajetan Bentele, Annelie Blumrich, Elia Smeir, Zsofia Ban, Sarah Brix, Jana Grune, Niklas Beyhoff, Robert Klopfleisch, Sebastian Dunst, Michal A Surma, Christian Klose, Michael Rothe, Frank R Heinzel, Alexander Krannich, Erin E Kershaw, Dieter Beule, P Christian Schulze, Nikolaus Marx, Ulrich Kintscher
Adipose tissue lipolysis occurs during the development of heart failure as a consequence of chronic adrenergic stimulation. However, the impact of enhanced adipose triacylglycerol hydrolysis mediated by adipose triglyceride lipase (ATGL) on cardiac function is unclear. To investigate the role of adipose tissue lipolysis during heart failure, we generated mice with tissue-specific deletion of ATGL (atATGL-KO). atATGL-KO mice were subjected to transverse aortic constriction (TAC) to induce pressure-mediated cardiac failure...
January 10, 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29319514/characterization-of-bacteriocin-producing-lactic-acid-bacteria-isolated-from-native-fruits-of-ecuadorian-amazon
#17
Karina Garzón, Clara Ortega, Gabriela N Tenea
Tropical, wild-type fruits are considered biodiverse "hotspots" of microorganisms with possible functional characteristics to be investigated. In this study, several native lactic acid bacteria (LAB) of Ecuadorian Amazon showing highly inhibitory potential were identified and characterized. Based on carbohydrate fermentation profile and 16S rRNA gene sequencing, seven strains were assigned as Lactobacillus plantarum and one strain as Weissella confusa. Using agar-well diffusion method the active synthetized components released in the neutralized and hydroxide peroxide eliminated cell-free supernatant were inhibited by proteolytic enzymes, while the activity was maintained stable after the treatment with catalase, lysozyme, α-amylase and lipase suggesting their proteinaceous nature...
December 4, 2017: Polish Journal of Microbiology
https://www.readbyqxmd.com/read/29317958/relationship-between-angiopoietin-like-protein-8-and-fasting-serum-triglyceride-level
#18
Hodaka Yamada, Ikuyo Kusaka, Rika Saikawa, Kazuo Hara, Masafumi Kakei, San-E Ishikawa
Background: The aim of the study was to evaluate the correlation between angiopoietin-like protein 8 (ANGPTL8) and metabolic parameters in non-diabetic healthy humans. Methods: We enrolled 30 healthy Japanese adults (25 men and five women). After 9 h of fasting, we collected blood samples and analyzed the ANGPTL8, lipoprotein lipase (LPL), plasma lipid and glucose metabolic parameters. In addition, we performed 75-g oral glucose tolerance test (OGTT) and measured adipokines (tumor necrosis factor-α, leptin and adiponectin)...
February 2018: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/29317208/zinc-alpha2-glycoprotein-promotes-browning-in-adipocytes
#19
Xin-Hua Xiao, Xiao-Yan Qi, Ya-Di Wang, Li Ran, Jing Yang, Huan-Li Zhang, Can-Xin Xu, Ge-Bo Wen, Jiang-Hua Liu
Recent studies have highlighted recruiting and activating brite adipocytes in WAT (so-called "browning") would be an attractive anti-obesity strategy. Zinc alpha2 glycoprotein (ZAG) is reported to ameliorate glycolipid metabolism and lose body weight in obese mice. However whether the body reducing effect mediated by browning programme remains unclear. Here, we show that overexpression of ZAG in 3T3-L1 adipocytes enhanced expression of brown fat-specific markers (UCP-1, PRDM16 and CIDEA), mitochondrial biogenesis genes (PGC-1α, NRF-1/2 and mtTFA) and the key lipid metabolism lipases (ATGL, HSL, CPT1-A and p-acyl-CoA carboxylase)...
January 6, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29315638/comparative-genomics-and-transcriptomics-depict-ericoid-mycorrhizal-fungi-as-versatile-saprotrophs-and-plant-mutualists
#20
Elena Martino, Emmanuelle Morin, Gwen-Aëlle Grelet, Alan Kuo, Annegret Kohler, Stefania Daghino, Kerrie W Barry, Nicolas Cichocki, Alicia Clum, Rhyan B Dockter, Matthieu Hainaut, Rita C Kuo, Kurt LaButti, Björn D Lindahl, Erika A Lindquist, Anna Lipzen, Hassine-Radhouane Khouja, Jon Magnuson, Claude Murat, Robin A Ohm, Steven W Singer, Joseph W Spatafora, Mei Wang, Claire Veneault-Fourrey, Bernard Henrissat, Igor V Grigoriev, Francis M Martin, Silvia Perotto
Some soil fungi in the Leotiomycetes form ericoid mycorrhizal (ERM) symbioses with Ericaceae. In the harsh habitats in which they occur, ERM plant survival relies on nutrient mobilization from soil organic matter (SOM) by their fungal partners. The characterization of the fungal genetic machinery underpinning both the symbiotic lifestyle and SOM degradation is needed to understand ERM symbiosis functioning and evolution, and its impact on soil carbon (C) turnover. We sequenced the genomes of the ERM fungi Meliniomyces bicolor, M...
January 7, 2018: New Phytologist
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