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https://www.readbyqxmd.com/read/29044175/a-skeletal-muscle-model-of-infantile-onset-pompe-disease-with-patient-specific-ips-cells
#1
Takeshi Yoshida, Tomonari Awaya, Tatsuya Jonouchi, Ryo Kimura, Shigemi Kimura, Takumi Era, Toshio Heike, Hidetoshi Sakurai
Pompe disease is caused by an inborn defect of lysosomal acid α-glucosidase (GAA) and is characterized by lysosomal glycogen accumulation primarily in the skeletal muscle and heart. Patients with the severe type of the disease, infantile-onset Pompe disease (IOPD), show generalized muscle weakness and heart failure in early infancy. They cannot survive over two years. Enzyme replacement therapy with recombinant human GAA (rhGAA) improves the survival rate, but its effect on skeletal muscle is insufficient compared to other organs...
October 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29042205/postnatal-administration-of-memantine-rescues-tnf-%C3%AE-induced-decreased-hippocampal-precursor-proliferation
#2
Zhongke Wang, Xie He, Xiaotang Fan
Pro-inflammatory cytokine exposure in early postnatal life triggers clear neurotoxic effects on the developing hippocampus. Tumor necrosis factor alpha (TNF-α) is one of the inflammatory mediators and is a potent inhibitor of neurogenesis. Memantine (MEM) is an uncompetitive N-methyl-D-aspartate (NMDA) receptor antagonist that has been demonstrated to increase the proliferation of hippocampal progenitor cells. However, the effects of MEM on TNF-α-mediated impairment of hippocampal precursor proliferation remain unclear...
October 14, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/29040870/parkin-absence-accelerates-microtubule-aging-in-dopaminergic-neurons
#3
Daniele Cartelli, Alida Amadeo, Alessandra Maria Calogero, Francesca Vittoria Marialuisa Casagrande, Carmelita De Gregorio, Mariarosa Gioria, Naoko Kuzumaki, Ilaria Costa, Jenny Sassone, Andrea Ciammola, Nobutaka Hattori, Hideyuki Okano, Stefano Goldwurm, Laurent Roybon, Gianni Pezzoli, Graziella Cappelletti
Loss-of-function caused by mutations in the parkin gene (PARK2) lead to early-onset familial Parkinson's disease. Recently, mechanistic studies proved the ability of parkin in regulating mitochondria homeostasis and microtubule (MT) stability. Looking at these systems during aging of PARK2 knockout mice, we found that loss of parkin induced an accelerated (over)acetylation of MT system both in dopaminergic neuron cell bodies and fibers, localized in the substantia nigra and corpus striatum, respectively. Interestingly, in PARK2 knockout mice, changes of MT stability preceded the alteration of mitochondria transport...
September 20, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29037799/thermography-as-an-early-predictive-measurement-for-evaluating-epidural-and-femoral-sciatic-block-success-in-dogs
#4
Nina Küls, Karen J Blissitt, Darren J Shaw, Gudrun Schöffmann, Richard E Clutton
OBJECTIVE: To evaluate skin temperature increase as an early predictive measure for evaluating epidural and femoral-sciatic block success in dogs. STUDY DESIGN: Prospective clinical trial. ANIMALS: A total of 29 dogs undergoing orthopaedic surgery on one hindlimb. METHODS: Dogs were anaesthetized and placed into lateral recumbency with the affected limb uppermost and the coat was clipped. Baseline infrared thermographic images (T0) of the affected limb, of the paw pad of the affected leg and of the ipsilateral paw pad were taken...
March 25, 2017: Veterinary Anaesthesia and Analgesia
https://www.readbyqxmd.com/read/29037558/monozygotic-twins-discordant-for-trisomy-21-discussion-of-etiological-events-involved
#5
Yao-Lung Chang, Wu-Pei Yi, An-Shine Chao, Kuan-Ju Chen, Po-Jen Cheng, Tzu-Hao Wang, Shuenn-Dyh Chang
OBJECTIVE: To elucidate the etiologies of discordant trisomy 21 in monozygotic twin pregnancy. CASE REPORT: A monochorionic diamniotic twin pregnancy with hydrops and cleft lip (twin 1) found in one fetus presented at gestational age of 17 weeks. Amniotic fluid karyotyping showed nonmosaic trisomy 21 in twin 1 (47, XY, +21 [20]) and a normal karyotype in twin 2 (46, XY [20]). Short tandem repeat (STR) polymorphism markers revealed that the two fetuses were monozygotic, and the two chromosomes 21 were maternal isodisomy in the trisomy fetus...
October 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29037085/luteal-phase-serum-progesterone-levels-after-gnrh-agonist-trigger-how-low-is-still-high-enough-for-an-ongoing-pregnancy
#6
B Lawrenz, S Samir, L Melado, F Ruiz, H M Fatemi
In the past years, individualization of assisted reproductive technique (ART)-treatment is increasingly common to customize the treatment protocol to the patient's specific conditions. The use of GnRH-agonist for final oocyte maturation in a gonadotropin-releasing hormone (GnRH)-antagonist protocol is the best approach to reduce the risk for ovarian hyperstimulation in high responder patients. However, due to severe luteolysis, the reproductive outcome with this approach in combination with the use of vaginal progesterone as luteal phase support, was poor...
October 16, 2017: Gynecological Endocrinology
https://www.readbyqxmd.com/read/29036437/fimbrins-4-and-5-act-synergistically-during-polarized-pollen-tube-growth-to-ensure-fertility-in-arabidopsis
#7
Hui Su, Hualing Feng, Xiaoting Chao, Xia Ding, Qiong Nan, Chenxi Wen, Huidong Liu, Sinuo Liu, Yun Xiang, Wenzhe Liu
The germination and polar growth of pollen are prerequisite for double fertilization in plants. The actin cytoskeleton and its binding proteins play pivotal roles in pollen germination and pollen-tube growth. Two homologs of the actin-bundling protein fimbrin, AtFIM4 and AtFIM5, are highly expressed in pollen in Arabidopsis and can form distinct actin architectures in vitro, but how they cooperatively regulate pollen germination and pollen-tube growth in vivo is largely unknown. In this study, we explored their functions during pollen germination and polar growth...
September 29, 2017: Plant & Cell Physiology
https://www.readbyqxmd.com/read/29030418/short-delay-to-initiate-plasma-exchange-is-the-strongest-predictor-of-outcome-in-severe-attacks-of-nmo-spectrum-disorders
#8
Mickael Bonnan, Rudy Valentino, Stéphane Debeugny, Harold Merle, Jean-Louis Fergé, Hossein Mehdaoui, Philippe Cabre
INTRODUCTION: Severe attacks of neuromyelitis optica spectrum disorder (NMO-SD) are improved by plasma exchange (PLEX) given as an adjunctive therapy. Initial studies failed to demonstrate a delay of PLEX treatment influenced clinical outcome; however PLEX was always used late. We examine the clinical consequences of delay in PLEX initiation on severe optic neuritis and spinal cord attacks in NMO-SD. METHODS: All of our patients who suffered attacks of NMO-SD, treated in our centre by PLEX, were retrospectively considered for inclusion...
October 13, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29024060/loss-of-dnmt3b-in-chondrocytes-leads-to-delayed-endochondral-ossification-and-fracture-repair
#9
Cuicui Wang, Yousef Abu-Amer, Regis J O'Keefe, Jie Shen
Despite advanced understanding of signaling mediated by local and systemic factors, the role of epigenetic factors in the regulation of bone regeneration remains vague. The DNA methyltransferases (Dnmts) Dnmt3a and Dnmt3b have tissue specific expression patterns and create unique methylation signatures to regulate gene expression. Using a stabilized murine tibia fracture model we find that Dnmt3b is induced early in fracture healing, peaks at 10 days post fracture (dpf), and declines to nearly undetectable levels by 28 dpf...
October 10, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29022864/reverse-genetic-system-genetically-stable-reporter-viruses-and-packaged-subgenomic-replicon-based-on-a-brazilian-zika-virus-isolate
#10
Margit Mutso, Sirle Saul, Kai Rausalu, Olga Susova, Eva Žusinaite, Suresh Mahalingam, Andres Merits
Zika virus (ZIKV, genus Flavivirus) has emerged as a major mosquito-transmitted human pathogen, with recent outbreaks associated with an increased incidence of neurological complications, particularly microcephaly and the Guillain-Barré syndrome. Because the virus has only very recently emerged as an important pathogen, research is being hampered by a lack of reliable molecular tools. Here we report an infectious cDNA (icDNA) clone for ZIKV isolate BeH819015 from Brazil, which was selected as representative of South American ZIKV isolated at early stages of the outbreak...
October 12, 2017: Journal of General Virology
https://www.readbyqxmd.com/read/29021278/dual-roles-of-glutathione-in-ecdysone-biosynthesis-and-antioxidant-function-during-the-larval-development-in-drosophila
#11
Sora Enya, Chikana Yamamoto, Hajime Mizuno, Tsuyoshi Esaki, Hsin-Kuang Lin, Masatoshi Iga, Kana Morohashi, Yota Hirano, Hiroshi Kataoka, Tsutomu Masujima, Yuko Shimada-Niwa, Ryusuke Niwa
Ecdysteroids, including the biologically active hormone 20-hydroxyecdysone (20E), play essential roles in controlling many developmental and physiological events in insects. Ecdysteroid biosynthesis is achieved by a series of specialized enzymes encoded in the Halloween genes. Recently, a new class of Halloween gene, noppera-bo (nobo), encoding a glutathione S-transferase (GST) in dipteran and lepidopteran species, has been identified and characterized. GSTs are well known to conjugate substrates with the reduced form of glutathione (GSH), a bioactive tripeptide composed of glutamate, cysteine, and glycine...
October 11, 2017: Genetics
https://www.readbyqxmd.com/read/29018564/achieving-comprehensive-disease-control-in-patients-with-early-and-established-rheumatoid-arthritis-treated-with-adalimumab-plus-methotrexate-versus-methotrexate-alone
#12
Edward C Keystone, Ferdinand C Breedveld, Désirée van der Heijde, Ronald F van Vollenhoven, Paul Emery, Josef S Smolen, Iain Sainsbury, Stefan Florentinus, Hartmut Kupper, Kun Chen, Arthur Kavanaugh
OBJECTIVE: To evaluate the achievement of comprehensive disease control (CDC) following 1 year of treatment with adalimumab+methotrexate versus methotrexate alone and whether early achievement of remission (at week 24 or 26) is associated with CDC at week 52 in patients with either early or established rheumatoid arthritis (RA). METHODS: Post hoc analyses were conducted in three clinical studies assessing treatment with adalimumab+methotrexate: DE019 (NCT00195702) enrolled patients with established RA who were methotrexate inadequate responders; OPTIMA (NCT00420927) and PREMIER (NCT00195663) enrolled methotrexate-naive patients with early RA...
2017: RMD Open
https://www.readbyqxmd.com/read/29017823/erythroblast-differentiation-at-spleen-in-q137e-mutant-ribosomal-protein-s19-gene-knock-in-c57bl-6j-mice
#13
Koji Yamanegi, Naoko Yamada, Keiji Nakasho, Hiroshi Nishiura
We recently found that erythroblast-like cells derived from human leukaemia K562 cells express C5a receptor (C5aR) and produce its antagonistic and agonistic ligand ribosomal protein S19 (RP S19) polymer, which is cross-linked between K122 and Q137 by tissue transglutaminases. RP S19 polymer binds to the reciprocal C5aRs on erythroblast-like cells and macrophage-like cells derived from human monocytic THP-1 cells and promotes differentiation into reticulocyte-like cells through enucleation in vitro. To examine the roles of RP S19 polymer in mouse erythropoiesis, we prepared Q137E mutant RP S19 gene knock-in C57BL/6J mice...
October 5, 2017: Immunobiology
https://www.readbyqxmd.com/read/29016855/enhanced-tau-pathology-via-ranbp9-and-hsp90-hsc70-chaperone-complexes
#14
Jung A Woo, Tian Liu, Xingyu Zhao, Courtney Trotter, Ksenia Yrigoin, Sara Cazzaro, Emilio De Narvaez, Hirah Khan, Richard Witas, Anusha Bukhari, Kamal Makati, Xinming Wang, Chad Dickey, David E Kang
Accumulation of amyloid β (Aβ) and tau represent the two major pathological hallmarks of Alzheimer's disease (AD). Despite the critical importance of Aβ accumulation as an early event in AD pathogenesis, multiple lines of evidence indicate that tau is required to mediate Aβ-induced neurotoxic signals in neurons. We have previously shown that the scaffolding protein Ran-binding protein 9 (RanBP9), which is highly elevated in brains of AD and AD mouse models, both enhances Aβ production and mediates Aβ-induced neurotoxicity...
October 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28992293/inducible-overexpression-of-runx1b-c-in-human-embryonic-stem-cells-blocks-early-hematopoiesis-from-mesoderm
#15
B Chen, Jiawen Teng, Hongwei Liu, X Pan, Y Zhou, Shu Huang, Mowen Lai, Guohui Bian, Bin Mao, Wencui Sun, Qiongxiu Zhou, Shengyong Yang, Tatsutoshi Nakahata, Feng Ma
RUNX1 is absolutely required for definitive hematopoiesis, but the function of RUNX1b/c, two isoforms of human RUNX1, is unclear. We established inducible RUNX1b/c-overexpressing human embryonic stem cell (hESC) lines, in which RUNX1b/c overexpression prevented the emergence of CD34+ cells from early stage, thereby drastically reducing the production of hematopoietic stem/progenitor cells. Simultaneously, the expression of hematopoiesis-related factors was downregulated. However, such blockage effect disappeared from day 6 in hESC/AGM-S3 cell co-cultures, proving that the blockage occurred before the generation of hemogenic endothelial cells...
August 1, 2017: Journal of Molecular Cell Biology
https://www.readbyqxmd.com/read/28986419/type-iii-interferon-is-a-critical-regulator-of-innate-antifungal-immunity
#16
Vanessa Espinosa, Orchi Dutta, Constance McElrath, Peicheng Du, Yun-Juan Chang, Bryan Cicciarelli, Amy Pitler, Ian Whitehead, Joshua J Obar, Joan E Durbin, Sergei V Kotenko, Amariliz Rivera
Type III interferons (IFN-λs) are the most recently found members of the IFN cytokine family and engage IFNLR1 and IL10R2 receptor subunits to activate innate responses against viruses. We have identified IFN-λs as critical instructors of antifungal neutrophil responses. Using Aspergillus fumigatus (Af) as a model to study antifungal immune responses, we found that depletion of CCR2(+) monocytes compromised the ability of neutrophils to control invasive fungal growth. Using an unbiased approach, we identified type I and III IFNs as critical regulators of the interplay between monocytes and neutrophils responding to Af We found that CCR2(+) monocytes are an important early source of type I IFNs that prime optimal expression of IFN-λ...
October 6, 2017: Science Immunology
https://www.readbyqxmd.com/read/28981369/the-elusive-path-of-brain-tissue-oxygenation-and-cerebral-perfusion-in-harness-hang-syncope-in-mountain-climbers
#17
Francesca Lanfranconi, Luca Pollastri, Giovanni Corna, Manuela Bartesaghi, Massimiliano Novarina, Alessandra Ferri, Giuseppe Andrea Miserocchi
Lanfranconi, Francesca, Luca Pollastri, Giovanni Corna, Manuela Bartesaghi, Massimiliano Novarina, Alessandra Ferri, and Giuseppe Andrea Miserocchi. The elusive path of brain tissue oxygenation and cerebral perfusion in harness hang syncope in mountain climbers. High Alt Med Biol. 18:000-000, 2017. AIM: Harness hang syncope (HHS) is a risk that specifically affects wide ranges of situations requiring safety harnesses in mountains. An irreversible orthostatic stasis could lead to death if a prompt rescue is not performed...
October 5, 2017: High Altitude Medicine & Biology
https://www.readbyqxmd.com/read/28981088/hsf4-regulates-lens-fiber-cell-differentiation-by-activating-p53-and-its-downstream-regulators
#18
Meng Gao, Yuwen Huang, Ling Wang, Mi Huang, Fei Liu, Shengjie Liao, Shanshan Yu, Zhaojing Lu, Shanshan Han, Xuebin Hu, Zhen Qu, Xiliang Liu, Tinsae Assefa Yimer, Lifang Yang, Zhaohui Tang, David Wan-Cheng Li, Mugen Liu
Cataract refers to opacities of the lens that impede the passage of light. Mutations in heat shock transcription factor 4 (HSF4) have been associated with cataract; however, the mechanisms regarding how mutations in HSF4 cause cataract are still obscure. In this study, we generated an hsf4 knockout zebrafish model using TALEN technology. The mutant zebrafish developed an early-onset cataract with multiple developmental defects in lens. The epithelial cells of the lens were overproliferated, resulting in the overabundance of lens fiber cells in hsf4(null) zebrafish lens...
October 5, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28974642/an-essential-role-for-igf2-in-cartilage-development-and-glucose-metabolism-during-postnatal-long-bone-growth
#19
Tomoya Uchimura, Judith M Hollander, Daisy S Nakamura, Zhiyi Liu, Clifford J Rosen, Irene Georgakoudi, Li Zeng
Postnatal bone growth involves a dramatic increase in length and girth. Intriguingly, this period of growth is independent of growth hormone and the underlying mechanism is poorly understood. Recently, an IGF2 mutation was identified in humans with early postnatal growth restriction. Here, we show that IGF2 is essential for longitudinal and appositional murine postnatal bone development, which involves proper timing of chondrocyte maturation and perichondrial cell differentiation and survival. Importantly, the Igf2 null mouse model does not represent a simple delay of growth but instead uncoordinated growth plate development...
October 1, 2017: Development
https://www.readbyqxmd.com/read/28974505/hematopoietic-stem-cell-transplantation-rescues-the-hematological-immunological-and-vascular-phenotype-in-dada2
#20
Hasan Hashem, Ashish R Kumar, Ingo Müller, Florian Babor, Robbert Bredius, Jignesh Dalal, Amy P Hsu, Steven M Holland, Dennis D Hickstein, Stephen Jolles, Robert Krance, Ghadir Sasa, Mervi Taskinen, Minna Koskenvuo, Janna Saarela, Joris van Montfrans, Keith Wilson, Barbara Bosch, Leen Moens, Michael Hershfield, Isabelle Meyts
Deficiency of adenosine deaminase 2 (DADA2) is caused by biallelic deleterious mutations in CECR1 DADA2 results in variable autoinflammation and vasculopathy (recurrent fevers, livedo reticularis, polyarteritis nodosa, lacunar ischemic strokes and intracranial hemorrhages), immunodeficiency and bone marrow failure. TNF-α blockade is the treatment of choice for the autoinflammation and vascular manifestations. Hematopoietic stem cell transplantation (HSCT) represents a potential definitive treatment. We present a cohort of 14 patients from 6 countries who received HSCT for DADA2...
October 3, 2017: Blood
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