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https://www.readbyqxmd.com/read/28549096/the-molecular-mechanisms-of-obesity-paradox
#1
Alexios S Antonopoulos, Dimitris Tousoulis
Clinical observations suggest a complex relationship between human obesity and cardiovascular disease. Whilst abdominal (visceral) adiposity leads to deleterious metabolic disturbances, subcutaneous fat accumulation has a benign effect on cardiometabolic risk. Notably, an accumulating body of evidence paradoxically links increased body mass index with a better prognosis in patients with established cardiovascular disease, a finding that has been termed the 'obesity paradox'. While this is now acknowledged to be an epidemiological finding, a metabolically healthy obese group associated with low cardiovascular risk has also been identified...
May 26, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28548876/the-constitutive-lipid-droplet-protein-plin2-regulates-autophagy-in-liver
#2
Tsung-Huang Tsai, Elaine Chen, Lan Li, Pradip Saha, Hsiao-Ju Lee, Li-Shin Huang, Gregory S Shelness, Lawrence Chan, Benny Hung-Junn Chang
Excess triglyceride (TG) accumulation in the liver underlies fatty liver disease, a highly prevalent ailment. TG occurs in the liver sequestered in lipid droplets, the major lipid storage organelle. Lipid droplets are home to the lipid droplet proteins, the most abundant of which are the perilipins (PLINs), encoded by 5 different genes, Plin1 to Plin5. Of the corresponding gene products, PLIN2 is the only constitutive and ubiquitously expressed lipid droplet protein that has been used as a protein marker for lipid droplets...
May 26, 2017: Autophagy
https://www.readbyqxmd.com/read/28545640/artificial-intelligence-in-precision%C3%A2-cardiovascular-medicine
#3
REVIEW
Chayakrit Krittanawong, HongJu Zhang, Zhen Wang, Mehmet Aydar, Takeshi Kitai
Artificial intelligence (AI) is a field of computer science that aims to mimic human thought processes, learning capacity, and knowledge storage. AI techniques have been applied in cardiovascular medicine to explore novel genotypes and phenotypes in existing diseases, improve the quality of patient care, enable cost-effectiveness, and reduce readmission and mortality rates. Over the past decade, several machine-learning techniques have been used for cardiovascular disease diagnosis and prediction. Each problem requires some degree of understanding of the problem, in terms of cardiovascular medicine and statistics, to apply the optimal machine-learning algorithm...
May 30, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28543881/a-review-of-blood-sample-handling-and-pre-processing-for-metabolomics-studies
#4
REVIEW
Vinicius Veri Hernandes, Coral Barbas, Danuta Dudzik
Metabolomics has been found to be applicable to a wide range of clinical studies, bringing a new era for improving clinical diagnostics, early disease detection, therapy prediction and treatment efficiency monitoring. A major challenge in metabolomics, particularly untargeted studies, is the extremely diverse and complex nature of biological specimens. Despite great advances in the field there still exist fundamental needs for considering pre-analytical variability that can introduce bias to the subsequent analytical process and decrease the reliability of the results and moreover confound final research outcomes...
May 19, 2017: Electrophoresis
https://www.readbyqxmd.com/read/28542051/critical-airway-stenosis-in-an-adolescent-male-with-pompe-disease-and-thoracic-lordosis-a-case-report
#5
B Randall Brenn, Mary T Theroux, Suken A Shah, William G Mackenzie, Robert Heinle, Mena T Scavina
An adolescent male with late-onset Pompe disease (glycogen storage disease type II) presented with a history of restrictive airway disease and a near-cardiorespiratory arrest during anesthesia for a liver biopsy initially thought to be due to bronchospasm. During a subsequent posterior spinal fusion procedure, he suffered cardiorespiratory arrest resulting in the procedure being aborted. Bronchoscopy performed shortly after resuscitation revealed an undiagnosed narrowing of the distal trachea and bronchi. This is the first description of a patient with lateonset Pompe disease with undiagnosed critical tracheal stenosis due to the progression of thoracic lordosis, which was ultimately relieved by posterior spinal fusion...
May 23, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28541437/hyperferritinemia-and-inflammation
#6
Kate Kernan, Joseph A Carcillo
Understanding of ferritin biology has traditionally centered on its role in iron storage and homeostasis, with low ferritin levels indicative of deficiency and high levels indicative of primary or secondary hemochromatosis. However, further work has shown that iron, redox biology and inflammation are inexorably linked. During infection, increased ferritin levels represent an important host defense mechanism that deprives bacterial growth of iron and protect immune cell function. It may also be protective, limiting the production of free radicals and mediating immunomodulation...
May 25, 2017: International Immunology
https://www.readbyqxmd.com/read/28541286/impaired-prosaposin-lysosomal-trafficking-in-frontotemporal-lobar-degeneration-due-to-progranulin-mutations
#7
Xiaolai Zhou, Lirong Sun, Oliver Bracko, Ji Whae Choi, Yan Jia, Alissa L Nana, Owen Adam Brady, Jean C Cruz Hernandez, Nozomi Nishimura, William W Seeley, Fenghua Hu
Haploinsufficiency of progranulin (PGRN) due to mutations in the granulin (GRN) gene causes frontotemporal lobar degeneration (FTLD), and complete loss of PGRN leads to a lysosomal storage disorder, neuronal ceroid lipofuscinosis (NCL). Accumulating evidence suggests that PGRN is essential for proper lysosomal function, but the precise mechanisms involved are not known. Here, we show that PGRN facilitates neuronal uptake and lysosomal delivery of prosaposin (PSAP), the precursor of saposin peptides that are essential for lysosomal glycosphingolipid degradation...
May 25, 2017: Nature Communications
https://www.readbyqxmd.com/read/28540636/gm2-activator-deficiency-caused-by-a-homozygous-exon-2-deletion-in-gm2a
#8
Patricia L Hall, Regina Laine, John J Alexander, Arunkanth Ankala, Lisa A Teot, Hart G W Lidov, Irina Anselm
GM2 activator (GM2A) deficiency (OMIM 613109) is a rare lysosomal storage disorder, with onset typically in infancy or early childhood. Clinically, it is almost indistinguishable from Tay-Sachs disease (OMIM 272800) or Sandhoff disease (OMIM 268800); however, traditionally available biochemical screening tests will most likely reveal normal results. We report a 2-year-old male with initially normal development until the age of 9 months, when he presented with developmental delay and regression. Workup at that time was unrevealing; at 15 months, he had abnormal brain MRI findings and a cherry red spot on ophthalmological examination...
May 25, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28540446/lipid-per-oxidation-in-mitochondria-an-emerging-target-in-the-ageing-process
#9
REVIEW
O S Ademowo, H K I Dias, D G A Burton, H R Griffiths
Lipids are essential for physiological processes such as maintaining membrane integrity, providing a source of energy and acting as signalling molecules to control processes including cell proliferation, metabolism, inflammation and apoptosis. Disruption of lipid homeostasis can promote pathological changes that contribute towards biological ageing and age-related diseases. Several age-related diseases have been associated with altered lipid metabolism and an elevation in highly damaging lipid peroxidation products; the latter has been ascribed, at least in part, to mitochondrial dysfunction and elevated ROS formation...
May 24, 2017: Biogerontology
https://www.readbyqxmd.com/read/28539924/therapeutic-development-of-mesenchymal-stem-cells-or-their-extracellular-vesicles-to-inhibit-autoimmune-mediated-inflammatory-processes-in-systemic-lupus-erythematosus
#10
REVIEW
Juhi Sharma, Jeffrey M Hampton, Giancarlo R Valiente, Takuma Wada, Holly Steigelman, Matthew C Young, Rachel R Spurbeck, Alisa D Blazek, Steffi Bösh, Wael N Jarjour, Nicholas A Young
Since being discovered over half a century ago, mesenchymal stem cells (MSCs) have been investigated extensively to characterize their cellular and physiological influences. MSCs have been shown to possess immunosuppressive capacity through inhibiting lymphocyte activation/proliferation and proinflammatory cytokine secretion while simultaneously demonstrating limited allogenic reactivity, which subsequently led to the evaluation of therapeutic feasibility to treat inflammatory diseases. Although regulatory constraints have restricted MSC development pharmacologically, limited clinical studies have shown encouraging results using MSC infusions to treat systemic lupus erythematosus (SLE); but, more trials will have to be performed to conclusively determine the clinical efficacy of MSCs to treat SLE...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28539676/results-from-polish-spondyloarthritis-initiative-registry-polspi-methodology-and-data-from-the-first-year-of-observation
#11
Zofia Guła, Tacjana Barczyńska, Marek Brzosko, Jerzy Gąsowski, Sławomir Jeka, Katarzyna Jodłowska-Cicio, Beata Kwaśny-Krochin, Piotr Leszczyński, Łukasz Lubiński, Katarzyna Łosińska, Katarzyna Pawlak-Buś, Hanna Przepiera-Będzak, Włodzimierz Samborski, Małgorzata Schlabs, Maciej Siedlar, Dorota Sikorska, Jerzy Świerkot, Małgorzata Węgierska, Piotr Wiland, Mariusz Korkosz
OBJECTIVES: Report on one-year results from the Polish Spondyloarthritis Initiative registry (PolSPI), containing the cross-sectional analysis of clinical and imaging data as well as database methodology. MATERIAL AND METHODS: The PolSPI registry includes patients with axial (axSpA) and peripheral (perSpA) spondyloarthritis according to ASAS classification criteria, and/or patients with ankylosing spondylitis according to modified New York criteria, psoriatic arthritis according to CASPAR criteria, arthropathy in inflammatory bowel disease, reactive arthritis, juvenile spondyloarthritis or undifferentiated spondyloarthritis...
2017: Reumatologia
https://www.readbyqxmd.com/read/28538700/stability-profiles-and-therapeutic-effect-of-cu-zn-superoxide-dismutase-chemically-coupled-to-o-quaternary-chitosan-derivatives-against-dextran-sodium-sulfate-induced-colitis
#12
Nan Zhao, Zhaolong Feng, Meng Shao, Jichao Cao, Fengshan Wang, Chunhui Liu
Superoxide dismutase (SOD) has attracted considerable attention on treatment of reactive oxygen species (ROS)-related disorders. We previously conjugated Cu/Zn SOD to O-quaternary chitosan derivatives (O-HTCC) to yield a polymer-enzyme conjugate O-HTCC-SOD that demonstrated superior therapeutic effect to native SOD. The present study demonstrated that O-HTCC-SOD had wider pH activity range, better thermal stability, excellent long-term stability for storage, as well as unique reinstatement of activity exposure to proteolytic degradation that was helpful for longer half-life in vivo...
May 24, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28538069/normocephalic-pancraniosynostosis-a-report-of-a-surgical-technique
#13
Marcin Czerwinski, Sharon Monsivais
Normocephalic pancraniosynostosis is defined as the premature fusion of 3 or, more major sutures in the absence of another primary etiology, including primary, microcephaly, ventriculoperitoneal shunting, hypothyroidism, rickets, mucopolysaccharidoses, or other lysosomal storage diseases. It is very rare, thus far only 6 patients have been reported in the literature. Patients tend to present much later than those with single sutural, synostoses, and up to half have evidence of elevated intracranial pressure...
May 19, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28535671/synergistic-effects-of-arginine-and-methyl-salicylate-on-alleviating-postharvest-disease-caused-by-botrysis-cinerea-in-tomato-fruit
#14
Xinhua Zhang, Dedong Min, Fujun Li, Nana Ji, Demei Meng, Ling Li
The effect of L-arginine (Arg, 1 mM) and methyl salicylate (MeSA, 0.05 mM) either separately or combined on gray mold caused by Botrytis cinerea in tomato fruit was investigated. The results showed that the application of Arg or MeSA alone could reduce gray mold and the combined treatment was more effective in inhibiting fungal decay during storage than each treatment separately. The combined treatment not only increased the activities of antioxidant enzymes such as superoxide dismutase, catalase and peroxidase, but also enhanced the gene expression levels of pathogenesis-related protein 1 and the activities of defense enzymes including phenylalanine ammonia-lyase, polyphenol oxidase, chitinase and β-1,3-glucanase during most of the storage periods, which were associated with lower disease incidence and disease index...
May 23, 2017: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/28534288/physicochemical-and-biological-characterization-of-sustained-isopropyl-unoprostone-release-device-made-of-poly-ethyleneglycol-dimethacrylates
#15
Nobuhiro Nagai, Yasuko Izumida, Yoshimasa Yamazaki, Hirokazu Kaji, Junichi Kawasaki, Matsuhiko Nishizawa, Toshiaki Abe
Transscleral drug delivery is becoming increasingly popular to manage posterior eye diseases. To evaluate the clinical application of a transscleral, sustained, unoprostone (UNO)-release device (URD) constructed of photopolymerized tri(ethyleneglycol) dimethacrylate and poly(ethyleneglycol) dimethacrylate, we evaluated physicochemical and biological properties of this device. The URD consists of a drug-impermeable reservoir and a semi-permeable cover. The in vitro release rate of UNO from the URD increased with increasing temperatures from 20 to 45 °C...
July 2017: Journal of Materials Science. Materials in Medicine
https://www.readbyqxmd.com/read/28533959/preservation-media-durations-and-cell-concentrations-of-short-term-storage-affect-key-features-of-human-adipose-derived-mesenchymal-stem-cells-for-therapeutic-application
#16
Fengli Zhang, Huaijuan Ren, Xiaohu Shao, Chao Zhuang, Yantian Chen, Nianmin Qi
BACKGROUND: Adipose-derived mesenchymal stem cells (ADSCs) have shown great potential in the treatment of various diseases. However, the optimum short-term storage condition of ADSCs in 2∼8 °C is rarely reported. This study aimed at optimizing a short-term storage condition to ensure the viability and function of ADSCs before transplantation. METHODS: Preservation media and durations of storage were evaluated by cell viability, apoptosis, adhesion ability and colony-forming unit (CFU) capacity of ADSCs...
2017: PeerJ
https://www.readbyqxmd.com/read/28532785/best-practice-in-the-measurement-and-interpretation-of-lysosomal-acid-lipase-in-dried-blood-spots-using-the-inhibitor-lalistat-2
#17
Zoltan Lukacs, Marianne Barr, John Hamilton
Lysosomal acid lipase deficiency (LAL-D) is an inherited, autosomal recessive lysosomal storage disorder characterized by progressive damage in multiple organ systems. Diagnosis is especially important in infants, in whom the course of disease is rapidly lethal without treatment. The recent regulatory approval of recombinant human lysosomal acid lipase (LAL), sebelipase alfa, merits rapid diagnosis in clinical routine, particularly in infants. A method for measuring LAL activity in dried blood spot (DBS) samples using the highly specific LAL inhibitor Lalistat 2 is available...
May 19, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28532689/lipid-composition-of-microdomains-is-altered-in-neuronopathic-gaucher-disease-sheep-brain-and-spleen
#18
Leanne K Hein, Tina Rozaklis, Melissa K Adams, John J Hopwood, Litsa Karageorgos
Gaucher disease is a lysosomal storage disorder caused by a deficiency in glucocerebrosidase activity that leads to accumulation of glucosylceramide and glucosylsphingosine. Membrane raft microdomains are discrete, highly organized microdomains with a unique lipid composition that provide the necessary environment for specific protein-lipid and protein-protein interactions to take place. In this study we purified detergent resistant membranes (DRM; membrane rafts) from the occipital cortex and spleen from sheep affected with acute neuronopathic Gaucher disease and wild-type controls...
May 17, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28531163/applications-of-gold-nanoparticles-in-nanomedicine-recent-advances-in-vaccines
#19
REVIEW
Sónia Alexandra Correia Carabineiro
Nowadays, gold is used in (nano-)medicine, usually in the form of nanoparticles, due to the solid proofs given of its therapeutic effects on several diseases. Gold also plays an important role in the vaccine field as an adjuvant and a carrier, reducing toxicity, enhancing immunogenic activity, and providing stability in storage. An even brighter golden future is expected for gold applications in this area.
May 22, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/28529827/deviant-lysosomal-ca-2-signalling-in-neurodegeneration-an-introduction
#20
Sandip Patel
Lysosomes are key acidic Ca(2+) stores. The principle Ca(2+)-permeable channels of the lysosome are TRP mucolipins (TRPMLs) and NAADP-regulated two-pore channels (TPCs). Recent studies, reviewed in this collection, have linked numerous neurodegenerative diseases to both gain and loss of function of TRPMLs/TPCs, as well as to defects in acidic Ca(2+) store content. These diseases span rare lysosomal storage disorders such as Mucolipidosis Type IV and Niemann-Pick disease, type C, through to more common ones such as Alzheimer and Parkinson disease...
June 1, 2016: Messenger
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