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https://www.readbyqxmd.com/read/28808947/the-sydney-heart-bank-improving-translational-research-while-eliminating-or-reducing-the-use-of-animal-models-of-human-heart-disease
#1
REVIEW
C G Dos Remedios, S P Lal, A Li, J McNamara, A Keogh, P S Macdonald, R Cooke, E Ehler, R Knöll, S B Marston, J Stelzer, H Granzier, C Bezzina, S van Dijk, F De Man, G J M Stienen, J Odeberg, F Pontén, W Linke, J van der Velden
The Sydney Heart Bank (SHB) is one of the largest human heart tissue banks in existence. Its mission is to provide high-quality human heart tissue for research into the molecular basis of human heart failure by working collaboratively with experts in this field. We argue that, by comparing tissues from failing human hearts with age-matched non-failing healthy donor hearts, the results will be more relevant than research using animal models, particularly if their physiology is very different from humans. Tissue from heart surgery must generally be used soon after collection or it significantly deteriorates...
August 14, 2017: Biophysical Reviews
https://www.readbyqxmd.com/read/28808590/genesis-and-maintenance-of-attentional-biases-the-role-of-the-locus-coeruleus-noradrenaline-system
#2
REVIEW
Mana R Ehlers, Rebecca M Todd
Emotionally arousing events are typically better remembered than mundane ones, in part because emotionally relevant aspects of our environment are prioritized in attention. Such biased attentional tuning is itself the result of associative processes through which we learn affective and motivational relevance of cues. We propose that the locus coeruleus-noradrenaline (LC-NA) system plays an important role in the genesis of attentional biases through associative learning processes as well as their maintenance...
2017: Neural Plasticity
https://www.readbyqxmd.com/read/28807406/longitudinal-changes-in-segmental-aortic-stiffness-determined-by-cardiac-magnetic-resonance-in-children-and-young-adults-with-connective-tissue-disorders-the-marfan-loeys-dietz-and-ehlers-danlos-syndromes-and-nonspecific-connective-tissue-disorders
#3
Anthony Merlocco, Ronald V Lacro, Kimberlee Gauvreau, Nicole Rabideau, Michael N Singh, Ashwin Prakash
Aortic stiffness measured by cardiac magnetic resonance (CMR) in connective tissue disorder (CTD) patients has been previously shown to be abnormal and to be associated with adverse aortic outcomes. The rate of increase in aortic stiffness with normal aging has been previously described. However, longitudinal changes in aortic stiffness have not been characterized in CTD patients. We examined longitudinal changes in CMR-derived aortic stiffness in children and young adults with CTDs. A retrospective analysis of 50 children and young adults (median age, 20 years; range, 0...
July 14, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28800066/treatment-of-the-first-acute-relapse-following-therapeutic-plasma-exchange-in-formerly-glucocorticosteroid-unresponsive-multiple-sclerosis-patients-a-multicenter-study-to-evaluate-glucocorticosteroid-responsiveness
#4
Johannes Ehler, Stephan Blechinger, Paulus S Rommer, Sebastian Koball, Steffen Mitzner, Hans-Peter Hartung, Fritz Leutmezer, Martin Sauer, Uwe K Zettl
Therapeutic options to treat multiple sclerosis (MS) relapses comprise glucocorticosteroids (GCS) as first-line and therapeutic plasma exchange (TPE) as second-line treatments in GCS-unresponsive patients. No guidelines exist for the treatment of another relapse following TPE. We retrospectively analyzed the responsiveness to GCS in a subsequent relapse following TPE in previously GCS-unresponsive MS patients. Thirty-seven patients with GCS-unresponsive MS relapses received TPE (relapse A). All patients developed another relapse after the completion of TPE and received GCS again (relapse B)...
August 11, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28795892/cold-atmospheric-pressure-plasma-for-treatment-of-chronic-wounds-drug-or-medical-device
#5
A Kramer, B R Conway, K Meissner, F Scholz, B H Rauch, A Moroder, A Ehlers, A J Meixner, C-D Heidecke, L I Partecke, M Kietzmann, O Assadian
OBJECTIVE: The use of cold atmospheric pressure plasma (CAPP) as a new therapeutic option to aid the healing of chronic wounds appears promising. Currently, uncertainty exists regarding their classification as medical device or medical drug. Because the classification of CAPP has medical, legal, and economic consequences as well as implications for the level of preclinical and clinical testing, the correct classification is not an academic exercise, but an ethical need. METHOD: A multidisciplinary team of physicians, surgeons, pharmacists, physicists and lawyers has analysed the physical and technical characteristics as well as legal conditions of the biological action of CAPP...
August 2, 2017: Journal of Wound Care
https://www.readbyqxmd.com/read/28790441/equilibrium-skyrmion-lattice-ground-state-in-a-polar-easy-plane-magnet
#6
S Bordács, A Butykai, B G Szigeti, J S White, R Cubitt, A O Leonov, S Widmann, D Ehlers, H-A Krug von Nidda, V Tsurkan, A Loidl, I Kézsmárki
The skyrmion lattice state (SkL), a crystal built of mesoscopic spin vortices, gains its stability via thermal fluctuations in all bulk skyrmion host materials known to date. Therefore, its existence is limited to a narrow temperature region below the paramagnetic state. This stability range can drastically increase in systems with restricted geometries, such as thin films, interfaces and nanowires. Thermal quenching can also promote the SkL as a metastable state over extended temperature ranges. Here, we demonstrate more generally that a proper choice of material parameters alone guarantees the thermodynamic stability of the SkL over the full temperature range below the paramagnetic state down to zero kelvin...
August 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28781928/posttraumatic-stress-disorder-and-autobiographical-memories-in-everyday-life
#7
Sabine Schönfeld, Anke Ehlers
Evidence from self-reports and laboratory studies suggests that recall of nontrauma autobiographical memories may be disturbed in posttraumatic stress disorder (PTSD), but investigations in everyday life are sparse. This study investigated unintentional nontrauma and trauma memories in trauma survivors with and without PTSD (N = 52), who kept an autobiographical memory diary for a week. We investigated whether unintentional nontrauma memories show an overgeneral memory bias and further memory abnormalities in people with PTSD, and whether unintentional trauma memories show distinct features...
March 2017: Clinical Psychological Science
https://www.readbyqxmd.com/read/28781834/ehlers-danlos-hypermobility-type-in-an-adult-with-chronic-pain-and-fatigue-a-case-study
#8
Sarah Cohen, Fred Markham
Ehlers-Danlos syndrome hypermobility type (EDS-HT) is an underdiagnosed genetic connective tissue disorder that causes joint hypermobility and widespread pain. We present a patient with the chief complaint of shoulder pain, a long history of widespread joint pain, and associated comorbidities. EDS-HT provided a unifying diagnosis and direction for management.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28779530/pharmacology-and-drug-development-in-rare-diseases-the-attractiveness-and-expertise-of-the-french-medical-pharmacology
#9
Joëlle Micallef, Pierre Boutouyrie, Olivier Blin
Developing drugs for rare disease can be challenging due to specific rare disease characteristics. The French Medical Pharmacology is structured and positioned to play a major role in Orphan Drug Research and Development due to the required expertise concentrated into pharmacology departments, exclusively implemented within the French University Hospitals, public hospitals that are linked to a medical school (and often a pharmacy school) with numerous INSERM or CNRS labelled research units. In addition, these structures allow a close collaboration between researchers, academic institutions and biotech start-up (most of them being spin-off of the academic structures)...
August 5, 2017: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/28769731/identification-key-to-nephtyidae-annelida-of-the-sea-of-okhotsk
#10
REVIEW
Inna L Alalykina, Nataliya Yu Dnestrovskaya, Igor A Jirkov
Currently, 15 species of Nephtyidae (Annelida) are known from the Sea of Okhotsk (north-western Pacific). A new user-friendly identification key is presented with a brief description for each species. The taxonomic positions of three closely related species, Nephtys brachycephala Moore, 1903, N. schmitti Hartman, 1938 and N. paradoxa Malm, 1874, are revised. The distributions of two species, Nephtys discors Ehlers, 1968 and N. assignis Hartman, 1950, are discussed.
2017: ZooKeys
https://www.readbyqxmd.com/read/28758664/structural-relaxation-viscosity-and-network-connectivity-in-a-hydrogen-bonding-liquid
#11
Stefania Perticaroli, Barmak Mostofian, Georg Ehlers, Joerg C Neuefeind, Souleymane O Diallo, Christopher B Stanley, Luke Daemen, Takeshi Egami, John Katsaras, Xiaolin Cheng, Jonathan D Nickels
In liquids, the ability of neighboring molecules to rearrange and jostle past each other is directly related to viscosity, the property which describes the propensity to flow. The presence of hydrogen bonds (H-bonds) complicates the molecular scale picture of viscosity. H-Bonds are attractive, directional interactions between molecules which, in some cases, result in transient network structures. In this work, we use experimental and computational methods to demonstrate that the timescale of H-bond network reorganization is the dominant dynamical timescale associated with viscosity for the case of the model H-bonding liquid n-methylacetamide (NMA)...
July 31, 2017: Physical Chemistry Chemical Physics: PCCP
https://www.readbyqxmd.com/read/28757364/ehlers-danlos-syndrome-kyphoscoliotic-type-due-to-lysyl-hydroxylase-1-deficiency-in-two-children-without-congenital-or-early-onset-kyphoscoliosis
#12
Fleur S van Dijk, Grazia M S Mancini, Alessandra Maugeri, Jan M Cobben
We report two children with Ehlers Danlos, kyphoscoliotic type confirmed by Lysyl Hydroxylase 1 deficiency due to bi-allelic PLOD1 mutations (kEDS-PLOD1) who were initially thought to have either a diagnosis of classical EDS (cEDS) or a neuromuscular disorder due to absence of (congenital) scoliosis. As the two patients reported here illustrate, patients with kEDS-PLOD1 do not always have a kyphoscoliosis present at birth or in the first year of life, neither do they necessarily develop kyphoscoliosis later in infancy...
July 27, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28752528/folic-acid-modulates-cancer-associated-micro-rnas-and-inflammatory-mediators-in-neoplastic-and-non-neoplastic-colonic-cells-in-a-different-way
#13
Birgit Niemann, Anke Nemitz, Josephine Werner, Ha Dong Mai, Pablo Steinberg, Alfonso Lampen, Anke Ehlers
SCOPE: Scientific evidence suggests that folic acid (FA) supplementation protects the healthy colonic mucosa from neoplastic transformation but may promote the progression of precancerous lesions. The underlying molecular mechanisms are not fully understood. Therefore, we explored, if high physiological FA doses provoke changes in (i) promoter-specific DNA methylation (ii) expression of cancer-associated micro RNAs (miRNAs) and (iii) inflammatory mediators in human neoplastic and non-neoplastic colonic cell lines...
July 28, 2017: Molecular Nutrition & Food Research
https://www.readbyqxmd.com/read/28742547/celiprolol-a-unique-selective-adrenoceptor-modulator
#14
James J Nawarskas, Angela Cheng-Lai, William H Frishman
Celiprolol is a β-blocker with a unique pharmacologic profile: it is a β1-andrenoceptor antagonist with partial β2 agonist activity. Given this combination of effects, celiprolol may be better described as a selective adrenoreceptor modulator. It has antihypertensive and antianginal properties and is indicated for those uses in various countries around the world. In the United States, however, the proposed indication for this drug will be for the treatment of vascular type Ehlers-Danlos syndrome, a rare connective tissue disorder characterized by fragile arterial structure and an increased risk of life-threatening vascular complications...
September 2017: Cardiology in Review
https://www.readbyqxmd.com/read/28742248/biallelic-col3a1-mutations-result-in-a-clinical-spectrum-of-specific-structural-brain-anomalies-and-connective-tissue-abnormalities
#15
Denise Horn, Eberhard Siebert, Ulrich Seidel, Imma Rost, Karin Mayer, Rami Abou Jamra, Diana Mitter, Uwe Kornak
Vascular Ehlers-Danlos syndrome (type IV) is an autosomal dominant disorder caused by heterozygous variants of COL3A1. We identified biallelic COL3A1 variants in two unrelated families. In a 3-year-old female with developmental delay the nonsense variant c.1282C>T, p.(Arg428*) was detected in combination the c.2057delC, p.(Pro686Leufs*105) frame shift variant. Both compound heterozygous variants were novel. This patient was born with bilateral clubfoot, joint laxity, and dysmorphic facial features. At the age of 2 years she developed an aneurysmal brain hemorrhage...
July 25, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28740411/subgroup-analysis-of-telehealthcare-for-patients-with-chronic-obstructive-pulmonary-disease-the-cluster-randomized-danish-telecare-north-trial
#16
Flemming Witt Udsen, Pernille H Lilholt, Ole K Hejlesen, Lars H Ehlers
PURPOSE: Results from the Danish cluster-randomized trial of telehealthcare to 1,225 patients with chronic obstructive pulmonary disease (COPD), the Danish Telecare North Trial, concluded that the telehealthcare solution was unlikely to be cost-effective, by applying international willingness-to-pay threshold values. The purpose of this article was to assess potential sources of variation across subgroups, which could explain overall cost-effectiveness results or be utilized in future economic studies in telehealthcare research...
2017: ClinicoEconomics and Outcomes Research: CEOR
https://www.readbyqxmd.com/read/28740084/ryanodine-receptors-are-part-of-the-myospryn-complex-in-cardiac-muscle
#17
Matthew A Benson, Caroline L Tinsley, Adrian J Waite, Francesca A Carlisle, Steve M M Sweet, Elisabeth Ehler, Christopher H George, F Anthony Lai, Enca Martin-Rendon, Derek J Blake
The Cardiomyopathy-associated gene 5 (Cmya5) encodes myospryn, a large tripartite motif (TRIM)-related protein found predominantly in cardiac and skeletal muscle. Cmya5 is an expression biomarker for a number of diseases affecting striated muscle and may also be a schizophrenia risk gene. To further understand the function of myospryn in striated muscle, we searched for additional myospryn paralogs. Here we identify a novel muscle-expressed TRIM-related protein minispryn, encoded by Fsd2, that has extensive sequence similarity with the C-terminus of myospryn...
July 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28739362/stroke-in-ehlers-danlos-syndrome-kyphoscoliotic-type-dissection-or-vasculitis
#18
Annegret Quade, Martin Wiesmann, Joachim Weis, Ingo Kurth, Houman Jalaie, Marianne Rohrbach, Martin Häusler
BACKGROUND: Patients with the kyphoscoliotic type of Ehlers-Danlos syndrome have an increased risk of vascular complications such as aortic dissection and perforation. Cerebral ischemia has only rarely been documented. PATIENT DESCRIPTION: This 13-year-old girl with the kyphoscoliotic type of Ehlers-Danlos syndrome had a large right middle cerebral artery distribution infarction. Full intravenous heparinization was started in response to presumed arterial dissection...
May 29, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28735213/chemoselective-synthesis-and-biological-evaluation-of-arylated-2-trifluoromethyl-quinolines-as-nucleotide-pyrophosphatase-npps-inhibitors
#19
David Kuhrt, Syeda Abida Ejaz, Saira Afzal, Shafi Ullah Khan, Joanna Lecka, Jean Sévigny, Peter Ehlers, Anke Spannenberg, Jamshed Iqbal, Peter Langer
A new approach to arylated 2-trifluoromethylquinolines based on novel regioselective Suzuki-Miyaura coupling reactions has been developed. Moreover, site-selective, chemo-selective amination reactions were performed. The new 2-trifluoromethylquinoline derivatives were tested as potential NPPs inhibitors and evaluated for their potential to inhibit two families of ecto-nucleotidases, i.e. NPPs and nucleoside triphosphate diphosphohydrolases (NTPDases). Several derivatives were active on a nanomolecular concentration...
July 13, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28734943/deficits-in-col5a2-expression-result-in-novel-skin-and-adipose-abnormalities-and-predisposition-to-aortic-aneurysms-and-dissections
#20
Arick C Park, Noel Phan, Dawiyat Massoudi, Zhenjie Liu, John F Kernien, Sheila M Adams, Jeffrey M Davidson, David E Birk, Bo Liu, Daniel S Greenspan
Classic Ehlers-Danlos syndrome (cEDS) is characterized by fragile, hyperextensible skin and hypermobile joints. cEDS can be caused by heterozygosity for missense mutations in genes COL5A2 and COL5A1, which encode the α2(V) and α1(V) chains, respectively, of collagen V, and is most often caused by COL5A1 null alleles. However, COL5A2 null alleles have yet to be associated with cEDS or other human pathologies. We previously showed that mice homozygous null for the α2(V) gene Col5a2 are early embryonic lethal, whereas haploinsufficiency caused aberrancies of adult skin, but not a frank cEDS-like phenotype, as skin hyperextensibility at low strain and dermal cauliflower-contoured collagen fibril aggregates, two cEDS hallmarks, were absent...
July 19, 2017: American Journal of Pathology
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