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Homers syndrome

Argyrios Tzouvelekis, Guoying Yu, Christian Lacks Lino Cardenas, Jose D Herazo-Maya, Rong Wang, Tony Woolard, Yi Zhang, Koji Sakamoto, Hojin Lee, Jae-Sung Yi, Giuseppe DeIuliis, Nikolaos Xylourgidis, Farida Ahangari, Patty J Lee, Vassilis Aidinis, Erica L Herzog, Robert Homer, Anton M Bennett, Naftali Kaminski
RATIONALE: Idiopathic Pulmonary Fibrosis (IPF) is a chronic fatal lung disease with dismal prognosis and no cure. The potential role of the ubiquitously expressed SH2 domain-containing-tyrosine phosphatase (SHP2) as a therapeutic target has not been studied in IPF Objectives: To determine the expression, mechanistic role and potential therapeutic utility of SHP2 in Pulmonary Fibrosis Methods: The effects of SHP2 overexpression and inhibition on fibroblast response to pro-fibrotic stimuli were analyzed in-vitro in primary human and mouse lung fibroblasts...
October 13, 2016: American Journal of Respiratory and Critical Care Medicine
Sureshbabu Angara, Mansoor Syed, Pragnya Das, Cecilia Janér, Gloria Pryhuber, Arshad Rahman, Sture Andersson, Robert J Homer, Vineet Bhandari
Administration of supplemental oxygen remains a critical clinical intervention for survival of preterm infants with respiratory failure. However, prolonged exposure to hyperoxia can augment pulmonary damage, resulting in developmental lung diseases embodied as hyperoxia-induced acute lung injury (HALI) and bronchopulmonary dysplasia (BPD). We sought to investigate the role of autophagy in hyperoxia-induced apoptotic cell death in developing lungs. We identified increased autophagy signaling in hyperoxia exposed MLE-12 cells, freshly-isolated fetal Type II alveolar epithelial cells, lungs of newborn (NB) wild-type (WT) mice, and human NB with respiratory distress syndrome and evolving and established BPD...
July 2, 2016: American Journal of Respiratory Cell and Molecular Biology
Weirui Guo, Gemma Molinaro, Katie A Collins, Seth A Hays, Richard Paylor, Paul F Worley, Karen K Szumlinski, Kimberly M Huber
UNLABELLED: Altered function of the Gq-coupled, Group 1 metabotropic glutamate receptors, specifically mGlu5, is implicated in multiple mouse models of autism and intellectual disability. mGlu5 dysfunction has been most well characterized in the fragile X syndrome mouse model, the Fmr1 knock-out (KO) mouse, where pharmacological and genetic reduction of mGlu5 reverses many phenotypes. mGlu5 is less associated with its scaffolding protein Homer in Fmr1 KO mice, and restoration of mGlu5-Homer interactions by genetic deletion of a short, dominant negative of Homer, H1a, rescues many phenotypes of Fmr1 KO mice...
February 17, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Shigeo Uchino, Chikako Waga
SHANK3 is a synaptic scaffolding protein and plays an important role in neuronal development. SHANK3 interacts with various synaptic molecules, including post-synaptic density-95 (PSD-95), homer and GluR1 AMPA receptor. SHANK3 gene is a causable gene of the Phelan- McDermid syndrome (also known as the 22q13.3 deletion syndrome), whose manifestation is global developmental delay and autistic behavior, especially shows severe speech and language deficit. Additionally since cumulative gene analysis in autistic subjects identified several mutations in SHANK3 gene, including deletion and duplication in a particular region, abnormality of SHANK3 gene is thought the be related with the neuropathology of autism spectrum disorder (ASD)...
2015: Current Neuropharmacology
Scott Mackenzie, Mark Nixon, Ashley Taylor, Rebecca Reynolds, Jennifer Bolton, Caroline Hayward, James Wilson, David Philips, Natalie Homer, Ruth Andrew, Brian Walker
No abstract text is available yet for this article.
November 2015: Psychoneuroendocrinology
Timothy D Folsom, Paul D Thuras, S Hossein Fatemi
Fragile X mental retardation protein (FMRP) is an RNA binding protein with 842 target mRNAs in mammalian brain. Silencing of the fragile X mental retardation 1 (FMR1) gene leads to loss of expression of FMRP and upregulated metabotropic glutamate receptor 5 (mGluR5) signaling resulting in the multiple physical and cognitive deficits associated with fragile X syndrome (FXS). Reduced FMRP expression has been identified in subjects with autism, schizophrenia, bipolar disorder, and major depression who do not carry the mutation for FMR1...
July 2015: Schizophrenia Research
Ai-Hui Tang, Bradley E Alger
The Fmr1 knock-out mouse model of fragile X syndrome (Fmr1(-/y)) has an epileptogenic phenotype that is triggered by group I metabotropic glutamate receptor (mGluR) activation. We found that a membrane-permeable peptide that disrupts mGluR5 interactions with long-form Homers enhanced mGluR-induced epileptiform burst firing in wild-type (WT) animals, replicating the early stages of hyperexcitability in Fmr1(-/y). The peptide enhanced mGluR-evoked endocannabinoid (eCB)-mediated suppression of inhibitory synapses, decreased it at excitatory synapses in WTs, but had no effect on eCB actions in Fmr1(-/y)...
March 4, 2015: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Vijay Shankar, Daniel Homer, Laura Rigsbee, Harry J Khamis, Sonia Michail, Michael Raymer, Nicholas V Reo, Oleg Paliy
The goal of this study was to determine if fecal metabolite and microbiota profiles can serve as biomarkers of human intestinal diseases, and to uncover possible gut microbe-metabolite associations. We employed proton nuclear magnetic resonance to measure fecal metabolites of healthy children and those diagnosed with diarrhea-predominant irritable bowel syndrome (IBS-D). Metabolite levels were associated with fecal microbial abundances. Using several ordination techniques, healthy and irritable bowel syndrome (IBS) samples could be distinguished based on the metabolite profiles of fecal samples, and such partitioning was congruent with the microbiota-based sample separation...
August 2015: ISME Journal
Marco Pignatelli, Sonia Piccinin, Gemma Molinaro, Luisa Di Menna, Barbara Riozzi, Milena Cannella, Marta Motolese, Gisella Vetere, Maria Vincenza Catania, Giuseppe Battaglia, Ferdinando Nicoletti, Robert Nisticò, Valeria Bruno
Angelman syndrome (AS) is caused by the loss of Ube3A, an ubiquitin ligase that commits specific proteins to proteasomal degradation. How this defect causes autism and other pathological phenotypes associated with AS is unknown. Long-term depression (LTD) of excitatory synaptic transmission mediated by type 5 metabotropic glutamate (mGlu5) receptors was enhanced in hippocampal slices of Ube3A(m-/p+) mice, which model AS. No changes were found in NMDA-dependent LTD induced by low-frequency stimulation. mGlu5 receptor-dependent LTD in AS mice was sensitive to the protein synthesis inhibitor anisomycin, and relied on the same signaling pathways as in wild-type mice, e...
March 26, 2014: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Samuel C Jurek, Mariko Hirano-Kobayashi, Homer Chiang, Daniel S Kohane, Benjamin D Matthews
The acute respiratory distress syndrome (ARDS), a devastating lung disease that has no cure, is exacerbated by life-supportive mechanical ventilation that worsens lung edema and inflammation through the syndrome of ventilator-induced lung injury. Recently, the membrane ion channel transient receptor potential vanilloid 4 (TRPV4) on alveolar macrophages was shown to mediate murine lung vascular permeability induced by high-pressure mechanical ventilation. The objective of this study was to determine whether inhalation of nanoparticles (NPs) containing the TRPV4 inhibitor ruthenium red (RR) prevents ventilator-induced lung edema in mice...
June 2014: American Journal of Respiratory Cell and Molecular Biology
Edward Passos, Brittany Dingley, Andrew Smith, Paul T Engels, Chad G Ball, Samir Faidi, Avery Nathens, Homer Tien
BACKGROUND: Haemorrhage in peripheral vascular injuries may cause life-threatening exsanguination. Tourniquets are used extensively by the military, with increased interest in the civilian setting to prevent deaths. This is a retrospective study of trauma patients at two large Canadian trauma centres with arterial injury after isolated extremity trauma. We hypothesized that tourniquet use may decrease mortality rate and transfusion requirements if applied early. METHODS: The study group was all adult patients at two Level 1 Trauma Centres in two Canadian cities in Canada, who had arterial injuries from extremity trauma...
March 2014: Injury
R E Ferner, J K Aronson
OBJECTIVE: To review the beneficial and harmful effects of laughter. DESIGN: Narrative synthesis. DATA SOURCES AND REVIEW METHODS: We searched Medline (1946 to June 2013) and Embase (1974 to June 2013) for reports of benefits or harms from laughter in humans, and counted the number of papers in each category. RESULTS: Benefits of laughter include reduced anger, anxiety, depression, and stress; reduced tension (psychological and cardiovascular); increased pain threshold; reduced risk of myocardial infarction (presumably requiring hearty laughter); improved lung function; increased energy expenditure; and reduced blood glucose concentration...
2013: BMJ: British Medical Journal
J-E Guillaume, P Gowreesunker
We report the case of a patient who developed a post-operative Homer's syndrome after non-cervical surgery. Part of the surgery was in the prone position. A hypothesis incriminating the prone position as being a possible cause of this syndrome is suggested.
2013: Acta Anaesthesiologica Belgica
A Guivarc'h-Levêque, F Jaffré, L Homer, L Moy, G Priou, D Colleu, I Denis, P Arvis
OBJECTIVE: To evaluate the efficacy and safety of ovulation triggering by agonists in antagonists IVF cycles with fresh embryo transfer in modulating low HCG dose for luteal phase support in patients at risk of ovarian hyperstimulation syndrome (OHSS). PATIENTS AND METHODS: In an observational study from September 2011 to March 2013, we triggered with agonist 107 cycles with OHSS risk, we initially triggered 39 cycles with 2 doses of Triptorelin 0.1 mg. Injection of 1500 IU HCG was performed one hour after the pick up and a second injection of 1500 IU was made 5 days later (group 1) combined with 400 mg of natural progesterone vaginally...
September 2013: Gynécologie, Obstétrique & Fertilité
Bartolomeu Nascimento, Jeannie Callum, Homer Tien, Gordon Rubenfeld, Ruxandra Pinto, Yulia Lin, Sandro Rizoli
BACKGROUND: Hemorrhage coupled with coagulopathy remains the leading cause of preventable in-hospital deaths among trauma patients. Use of a transfusion protocol with a predefined ratio of 1:1:1 (1 each of red blood cells [RBC], frozen plasma [FP] and platelets) has been associated with improved survival in retrospective studies in military and civilian settings, but such a protocol has its challenges and may increase the risk of respiratory complications. We conducted a randomized controlled trial to assess the feasibility of a 1:1:1 transfusion protocol and its effect on mortality and complications among patients with severe trauma...
September 3, 2013: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
F Lang, P Münzer, M Gawaz, O Borst
Platelet secretion and aggregation as well as thrombus formation of blood platelets critically depend on increase of cytosolic Ca2+ concentration ([Ca2+]i) mainly resulting from intracellular Ca2+ release followed by store operated Ca2+ entry (SOCE) through Ca2+ release activated channels (CRAC). SOCE is in part accomplished by the pore forming unit Orai and its regulator stromal interaction molecule (STIM). Orai1 and STIM1 transcription is stimulated by NF-κB (nuclear factor kappa B). Serum- and glucocorticoid-inducible kinase 1 (SGK1) up-regulates NF-κB-activity in megakaryocytes and thus Orai1-expression and SOCE in platelets...
November 2013: Thrombosis and Haemostasis
S Hossein Fatemi, Timothy D Folsom, Rachel E Kneeland, Mahtab K Yousefi, Stephanie B Liesch, Paul D Thuras
BACKGROUND: Candidate genes associated with idiopathic forms of autism overlap with other disorders including fragile X syndrome. Our laboratory has previously shown reduction in fragile X mental retardation protein (FMRP) and increase in metabotropic glutamate receptor 5 (mGluR5) in cerebellar vermis and superior frontal cortex (BA9) of individuals with autism. METHODS: In the current study we have investigated expression of four targets of FMRP and mGluR5 signaling - homer 1, amyloid beta A4 precursor protein (APP), ras-related C3 botulinum toxin substrate 1 (RAC1), and striatal-enriched protein tyrosine phosphatase (STEP) - in the cerebellar vermis and superior frontal cortex (BA9) via SDS-PAGE and western blotting...
2013: Molecular Autism
Senthy V Sellaturay, Raj Nair, Ian K Dickinson, Seshadri Sriprasad
AIMS: It is common knowledge that proteus bacteria are associated with urinary tract infections and urinary stones. Far more interesting however, is the derivation of the word proteus. This study examines the origin of the word proteus, its mythological, historical and literary connections and evolution to present-day usage. MATERIALS AND METHODS: A detailed search for primary and secondary sources was undertaken using the library and internet. RESULTS: Greek mythology describes Proteus as an early sea-god, noted for being versatile and capable of assuming many different forms...
October 2012: Indian Journal of Urology: IJU: Journal of the Urological Society of India
Romana Höftberger, Lidia Sabater, Angel Ortega, Josep Dalmau, Francesc Graus
IMPORTANCE: Homer proteins are a family of scaffolding proteins of the postsynaptic density. Homer-3 colocalizes and modulates the activity of group I metabotropic glutamate receptors (mGluR1 and mGluR5). Cerebellitis has been reported in association with antibodies to mGluR1. We describe the second patient with cerebellitis and Homer-3 antibodies and report a novel, highly specific immunoblot assay. OBSERVATIONS: A 38-year-old man had acute onset of headache, nausea, vomiting, and confusion...
April 2013: JAMA Neurology
Naiem Nassiri, Radhika Kapoor, Khalil Qato, Jiri Vitek, Robert J Rosen, Homere Al Moutran, Peter D Costantino, Richard M Green
Carotid blowout syndrome is a rare life-threatening complication of head and neck malignancy. Current literature has focused exclusively on the carotid system and associated branches. We present a unique case of multivessel blowout in the setting of a large nonresectable neck sarcoma requiring various endovascular techniques for palliation.
January 2013: Annals of Vascular Surgery
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