keyword
MENU ▼
Read by QxMD icon Read
search

fires syndrome

keyword
https://www.readbyqxmd.com/read/27910058/gene-delivery-for-the-generation-of-bioartificial-pacemaker
#1
Patrick K W Chan, Ronald A Li
Electronic pacemakers have been used in patients with heart rhythm disorders for device-supported pacing. While effective, there are such shortcomings as limited battery life, permanent implantation of catheters, the lack of autonomic neurohumoral responses, and risks of lead dislodging. Here we describe protocols for establishing porcine models of sick sinus syndrome and complete heart block, and the generation of bioartificial pacemaker by delivering a strategically engineered form of hyperpolarization-activated cyclic nucleotide-gated pacemaker channel protein via somatic gene transfer to convert atrial or ventricular muscle cardiomyocytes into nodal-like cells that rhythmically fire action potentials...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27864337/connexin-mediated-signalling-in-non-sensory-cells-is-crucial-for-the-development-of-sensory-inner-hair-cells-in-the-mouse-cochlea
#2
Stuart L Johnson, Federico Ceriani, Oliver Houston, Roman Polishchuk, Elena Polishchuk, Giulia Crispino, Veronica Zorzi, Fabio Mammano, Walter Marcotti
: Mutations in the genes encoding for gap junction proteins connexin 26 (Cx26) and connexin 30 (Cx30) have been linked to syndromic and non-syndromic hearing loss in mice and humans. The release of ATP from connexin hemichannels in cochlear non-sensory cells has been proposed to be the main trigger for action potential (AP) activity in immature sensory inner hair cells (IHCs), which is crucial for the refinement of the developing auditory circuitry. Using connexin knockout mice, we show that IHCs fire spontaneous APs even in the absence of ATP-dependent intercellular Ca(2+) signalling in the non-sensory cells...
November 18, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27856530/diagnostic-conundrum-lessons-from-the-man-who-accidently-set-fire-to-his-home
#3
Sahan Benedict Mendis, Victoria Lukats
A 62-year-old man presented to psychiatric services with a 3-month history of a range of symptoms which included obsessional thoughts, self-neglect, lack of mental flexibility, reduced ability to plan, organise and follow instructions, reduced capacity to empathise and disinhibition. He also accidently set fire to his house. Overall these findings are compatible with a dysexecutive syndrome. This man has a significant history of polysubstance misuse and chronic hepatitis C infection. Neuroimaging revealed an acquired traumatic brain injury which could account for his dysexecutive syndrome...
November 17, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27855662/microarray-expression-profile-analysis-of-mrnas-and-long-non-coding-rnas-in-pulmonary-tuberculosis-with-different-traditional-chinese-medicine-syndromes
#4
Ting-Ting Jiang, Li-Liang Wei, Li-Ying Shi, Zhong-Liang Chen, Chong Wang, Chang-Ming Liu, Zhong-Jie Li, Ji-Cheng Li
BACKGROUND: Combination chemotherapy with Western anti-tuberculosis (TB) drugs is the mainstay of TB treatment. Chinese herbal medicines with either heat clearing and detoxifying effects or nourishing Yin and reducing fire effects have been used to treat TB based on the Traditional Chinese Medicine (TCM) syndromes of TB patients. This study analyzed the expression profiles of long non-coding RNAs (lncRNAs) and mRNAs in TB patients with different TCM syndromes. METHODS: TB patients were classified as pulmonary Yin deficiency (PYD) syndrome, hyperactivity of fire due to Yin deficiency (HFYD) syndrome, and deficiency of Qi and Yin (DQY) syndrome...
November 17, 2016: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/27805592/in-vitro-recording-of-mesenteric-afferent-nerve-activity-in-mouse-jejunal-and-colonic-segments
#5
Sara Nullens, Annemie Deiteren, Wen Jiang, Christopher Keating, Hannah Ceuleers, Sven Francque, David Grundy, Joris G De Man, Benedicte Y De Winter
Afferent nerves not only convey information concerning normal physiology, but also signal disturbed homeostasis and pathophysiological processes of the different organ systems from the periphery towards the central nervous system. As such, the increased activity or 'sensitization' of mesenteric afferent nerves has been allocated an important role in the pathophysiology of visceral hypersensitivity and abdominal pain syndromes. Mesenteric afferent nerve activity can be measured in vitro in an isolated intestinal segment that is mounted in a purpose-built organ bath and from which the splanchnic nerve is isolated, allowing researchers to directly assess nerve activity adjacent to the gastrointestinal segment...
October 25, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27770579/febrile-infection-related-epilepsy-syndrome-treated-with-anakinra
#6
Daniel L Kenney-Jung, Annamaria Vezzani, Robert J Kahoud, Reghann G LaFrance-Corey, Mai-Lan Ho, Theresa Wampler Muskardin, Elaine C Wirrell, Charles L Howe, Eric T Payne
Febrile infection-related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and an unclear etiology. Anakinra is a recombinant version of the human interleukin-1 receptor antagonist used to treat autoinflammatory disorders. This is the first report of anakinra for treatment of a child with super-refractory status epilepticus secondary to FIRES. Anakinra was well tolerated and effective. Cerebral spinal fluid analysis revealed elevated levels of proinflammatory cytokines before treatment that normalized on anakinra, suggesting a potential pathogenic role for neuroinflammation in FIRES...
October 22, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27767173/euchromatin-histone-methyltransferase-1-regulates-cortical-neuronal-network-development
#7
Marijn Bart Martens, Monica Frega, Jessica Classen, Lisa Epping, Elske Bijvank, Marco Benevento, Hans van Bokhoven, Paul Tiesinga, Dirk Schubert, Nael Nadif Kasri
Heterozygous mutations or deletions in the human Euchromatin histone methyltransferase 1 (EHMT1) gene cause Kleefstra syndrome, a neurodevelopmental disorder that is characterized by autistic-like features and severe intellectual disability (ID). Neurodevelopmental disorders including ID and autism may be related to deficits in activity-dependent wiring of brain circuits during development. Although Kleefstra syndrome has been associated with dendritic and synaptic defects in mice and Drosophila, little is known about the role of EHMT1 in the development of cortical neuronal networks...
October 21, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27753838/sy-06-1-obesity-diabetes-and-hypertension-are-adipokines-responsible
#8
Anthony Heagerty
There is now a considerable body of evidence to suggest that the fat cells that surround blood vessels (perivascular adipose tissue, PVAT) can influence profoundly arterial tone by releasing vasodilator adipokines which can act locally in a paracrine fashion. In healthy lean individuals the primary vasodilator released appears to be adiponectin and there is a complex interation between autonomic nerve firing in PVAT and the release of nitric oxide from adipocytes and an increased bioavailability of adiponectin...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27704337/modeling-the-differentiation-of-a-and-c-type-baroreceptor-firing-patterns
#9
Jacob Sturdy, Johnny T Ottesen, Mette S Olufsen
The baroreceptor neurons serve as the primary transducers of blood pressure for the autonomic nervous system and are thus critical in enabling the body to respond effectively to changes in blood pressure. These neurons can be separated into two types (A and C) based on the myelination of their axons and their distinct firing patterns elicited in response to specific pressure stimuli. This study has developed a comprehensive model of the afferent baroreceptor discharge built on physiological knowledge of arterial wall mechanics, firing rate responses to controlled pressure stimuli, and ion channel dynamics within the baroreceptor neurons...
October 5, 2016: Journal of Computational Neuroscience
https://www.readbyqxmd.com/read/27703430/early-impairment-of-synaptic-and-intrinsic-excitability-in-mice-expressing-als-dementia-linked-mutant-ubqln2
#10
Daniel Radzicki, Erdong Liu, Han-Xiang Deng, Teepu Siddique, Marco Martina
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are believed to represent the different outcomes of a common pathogenic mechanism. However, while researchers have intensely studied the involvement of motor neurons in the ALS/FTD syndrome, very little is known about the function of hippocampal neurons, although this area is critical for memory and other cognitive functions. We investigated the electrophysiological properties of CA1 pyramidal cells in slices from 1 month-old UBQLN2(P497H) mice, a recently generated model of ALS/FTD that shows heavy depositions of ubiquilin2-positive aggregates in this brain region...
2016: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/27688999/mechanisms-underlying-prorenin-actions-on-hypothalamic-neurons-implicated-in-cardiometabolic-control
#11
Soledad Pitra, Yumei Feng, Javier E Stern
BACKGROUND: Hypertension and obesity are highly interrelated diseases, being critical components of the metabolic syndrome. Despite the growing prevalence of this syndrome in the world population, efficient therapies are still missing. Thus, identification of novel targets and therapies are warranted. An enhanced activity of the hypothalamic renin-angiotensin system (RAS), including the recently discovered prorenin (PR) and its receptor (PRR), has been implicated as a common mechanism underlying aberrant sympatho-humoral activation that contributes to both metabolic and cardiovascular dysregulation in the metabolic syndrome...
October 2016: Molecular Metabolism
https://www.readbyqxmd.com/read/27670841/hyperexcitability-of-mesencephalic-trigeminal-neurons-and-reorganization-of-ion-channel-expression-in-a-rett-syndrome-model
#12
Max F Oginsky, Ningren Cui, Weiwei Zhong, Christopher M Johnson, Chun Jiang
People with Rett syndrome (RTT) have defects in motor function also seen in Mecp2-null mice. Motor function depends on not only central motor commands but also sensory feedback that is vulnerable to changes in excitability of propriosensory neurons. Here we report evidence for hyperexcitability of mesencephalic trigeminal (Me5) neurons in Mecp2-null mice and a novel cellular mechanism for lowering its impact. In in-vitro brain slices, the Me5 neurons in both Mecp2(-/Y) male and symptomatic Mecp2(+/-) female mice were overly excitable showing increased firing activity in comparison to their wild-type (WT) male and asymptomatic counterparts...
September 27, 2016: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/27642933/sy-06-1-obesity-diabetes-and-hypertension-are-adipokines-responsible
#13
Anthony Heagerty
There is now a considerable body of evidence to suggest that the fat cells that surround blood vessels (perivascular adipose tissue, PVAT) can influence profoundly arterial tone by releasing vasodilator adipokines which can act locally in a paracrine fashion. In healthy lean individuals the primary vasodilator released appears to be adiponectin and there is a complex interation between autonomic nerve firing in PVAT and the release of nitric oxide from adipocytes and an increased bioavailability of adiponectin...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27638073/acute-unilateral-loss-of-vestibular-function
#14
M Fetter
Sudden unilateral loss of vestibular function is the most severe condition that can occur in the vestibular system. The clinical syndrome is caused by the physiologic properties of the vestibulo-ocular reflex (VOR) arc. In the normal situation, the two peripheral vestibular end organs are connected to a functional unit in coplanar pairs of semicircular canals working in a push-pull mode. "Push-pull" mode means that, when one side is excited, the other side is inhibited, and vice versa due to two mechanisms...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27598514/scn10a-mutation-in-a-patient-with-erythromelalgia-enhances-c-fiber-activity-dependent-slowing
#15
Andreas M Kist, Dagrun Sagafos, Anthony M Rush, Cristian Neacsu, Esther Eberhardt, Roland Schmidt, Lars Kristian Lunden, Kristin Ørstavik, Luisa Kaluza, Jannis Meents, Zhiping Zhang, Thomas Hedley Carr, Hugh Salter, David Malinowsky, Patrik Wollberg, Johannes Krupp, Inge Petter Kleggetveit, Martin Schmelz, Ellen Jørum, Angelika Lampert, Barbara Namer
Gain-of-function mutations in the tetrodotoxin (TTX) sensitive voltage-gated sodium channel (Nav) Nav1.7 have been identified as a key mechanism underlying chronic pain in inherited erythromelalgia. Mutations in TTX resistant channels, such as Nav1.8 or Nav1.9, were recently connected with inherited chronic pain syndromes. Here, we investigated the effects of the p.M650K mutation in Nav1.8 in a 53 year old patient with erythromelalgia by microneurography and patch-clamp techniques. Recordings of the patient's peripheral nerve fibers showed increased activity dependent slowing (ADS) in CMi and less spontaneous firing compared to a control group of erythromelalgia patients without Nav mutations...
2016: PloS One
https://www.readbyqxmd.com/read/27586296/unusual-voltage-gated-sodium-currents-as-targets-for-pain
#16
C Barbosa, T R Cummins
Pain is a serious health problem that impacts the lives of many individuals. Hyperexcitability of peripheral sensory neurons contributes to both acute and chronic pain syndromes. Because voltage-gated sodium currents are crucial to the transmission of electrical signals in peripheral sensory neurons, the channels that underlie these currents are attractive targets for pain therapeutics. Sodium currents and channels in peripheral sensory neurons are complex. Multiple-channel isoforms contribute to the macroscopic currents in nociceptive sensory neurons...
2016: Current Topics in Membranes
https://www.readbyqxmd.com/read/27559138/hypoxia-and-hypercapnia-inhibit-hypothalamic-orexin-neurons-in-rats
#17
Olga Dergacheva, Akihiro Yamanaka, Alan R Schwartz, Vsevolod Y Polotsky, David Mendelowitz
Evidence of impaired function of orexin neurons has been found in individuals with cardiorespiratory disorders, such as obstructive sleep apnea (OSA) and sudden infant death syndrome (SIDS), but the mechanisms responsible are unknown. Individuals with OSA and SIDS experience repetitive breathing cessations and/or rebreathing of expired air, resulting in hypoxia/hypercapnia (H/H). In this study, we examined the responses of fluorescently identified rat orexin neurons in the lateral hypothalamus to acute H/H to test if and how these neurons alter their activity and function during this challenge...
November 1, 2016: Journal of Neurophysiology
https://www.readbyqxmd.com/read/27538648/cytokine-related-and-sodium-channel-polymorphism-as-candidate-predisposing-factors-for-childhood-encephalopathy-fires-aerrps
#18
M Saitoh, K Kobayashi, I Ohmori, Y Tanaka, K Tanaka, T Inoue, A Horino, K Ohmura, A Kumakura, Y Takei, S Hirabayashi, M Kajimoto, T Uchida, S Yamazaki, T Shiihara, T Kumagai, M Kasai, H Terashima, M Kubota, M Mizuguchi
Febrile infection-related epilepsy syndrome (FIRES), or acute encephalitis with refractory, repetitive partial seizures (AERRPS), is an epileptic encephalopathy beginning with fever-mediated seizures. The etiology remains unclear. To elucidate the genetic background of FIRES/AERRPS (hereafter FIRES), we recruited 19 Japanese patients, genotyped polymorphisms of the IL1B, IL6, IL10, TNFA, IL1RN, SCN1A and SCN2A genes, and compared their frequency between the patients and controls. For IL1RN, the frequency of a variable number of tandem repeat (VNTR) allele, RN2, was significantly higher in the patients than in controls (p=0...
September 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27499081/loss-and-gain-of-mecp2-cause-similar-hippocampal-circuit-dysfunction-that-is-rescued-by-deep-brain-stimulation-in-a-rett-syndrome-mouse-model
#19
Hui Lu, Ryan T Ash, Lingjie He, Sara E Kee, Wei Wang, Dinghui Yu, Shuang Hao, Xiangling Meng, Kerstin Ure, Aya Ito-Ishida, Bin Tang, Yaling Sun, Daoyun Ji, Jianrong Tang, Benjamin R Arenkiel, Stelios M Smirnakis, Huda Y Zoghbi
Loss- and gain-of-function mutations in methyl-CpG-binding protein 2 (MECP2) underlie two distinct neurological syndromes with strikingly similar features, but the synaptic and circuit-level changes mediating these shared features are undefined. Here we report three novel signs of neural circuit dysfunction in three mouse models of MECP2 disorders (constitutive Mecp2 null, mosaic Mecp2(+/-), and MECP2 duplication): abnormally elevated synchrony in the firing activity of hippocampal CA1 pyramidal neurons, an impaired homeostatic response to perturbations of excitatory-inhibitory balance, and decreased excitatory synaptic response in inhibitory neurons...
August 17, 2016: Neuron
https://www.readbyqxmd.com/read/27465362/sensory-hypo-excitability-in-a-rat-model-of-fetal-development-in-fragile-x-syndrome
#20
Julia Berzhanskaya, Marnie A Phillips, Jing Shen, Matthew T Colonnese
Fragile X syndrome (FXS) is characterized by sensory hyper-sensitivity, and animal models suggest that neuronal hyper-excitability contributes to this phenotype. To understand how sensory dysfunction develops in FXS, we used the rat model (FMR-KO) to quantify the maturation of cortical visual responses from the onset of responsiveness prior to eye-opening, through age equivalents of human juveniles. Rather than hyper-excitability, visual responses before eye-opening had reduced spike rates and an absence of early gamma oscillations, a marker for normal thalamic function at this age...
2016: Scientific Reports
keyword
keyword
68765
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"