Breathing disturbances in a model of Rett syndrome: A potential involvement of the glycine receptor α3 subunit?

Abstract:

:The glycine receptor α3 subunit is known to be a target for cAMP/PKA-mediated phosphorylation and regulation. Mice that lack this subunit are apparently normal but the 5-HT1A-receptor mediated modulation of respiratory network activity is disturbed. Since the intracellular cAMP-concentration is reduced in mice that lack the transcriptional modulator methyl-CpG-binding protein 2 (MeCP2) gene, we aimed to test if the α3 subunit of the glycine receptor is involved in the development of the breathing phenotype of MeCP2-deficient mice (Mecp2-/y). Therefore, we generated a double knock-out mouse line that lacks both the Mecp2 gene as well as the gene (Glra3) for the α3 subunit of the ionotropic glycine receptor. As compared to WT and Glra3-/- mice, both Mecp2-/y mice and Mecp2-/y; Glra3-/- mice (DKO) showed a slower respiratory rate and a tendency towards higher numbers of apneas. Interestingly, the irregularity of the breathing was significantly reduced in DKO as compared to Mecp2-/y littermates. In the light of the unaltered survival of DKO mice, however, the contribution of the glycine receptor α3 subunit for development and progression of the breathing disturbances in the mouse model of Rett syndrome appears to be only of minor relevance.

authors

Mesuret G,Dannenberg J,Arnoldt M,Grützner AA,Niebert M,Hülsmann S

doi

10.1016/j.resp.2017.11.011

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

43-47

eissn

1569-9048

issn

1878-1519

pii

S1569-9048(17)30342-7

journal_volume

248

pub_type

杂志文章
  • Morphological differences of the carotid body among C57/BL6 (B6), A/J, and CSS B6A1 mouse strains.

    abstract::The C57/BL6 (B6) mouse strain exhibits post-hypoxic frequency decline and periodic breathing, as well as greater amount of irregular breathing during rest in comparison to the A/J and to the B6a1, a chromosomal substitution strain whereby the A/J chromosome 1 is bred onto the B6 background (Han et al., 2002; Yamauchi ...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2011.04.021

    authors: Chai S,Gillombardo CB,Donovan L,Strohl KP

    更新日期:2011-08-15 00:00:00

  • Bronchial responsiveness in an elastase-induced mouse model of emphysema.

    abstract::Bronchial responsiveness during methacholine (MCh) challenge was analysed in an elastase-induced mouse model of emphysema to explore the magnitude of the response in this model. Swiss mice were intratracheally instilled with saline or elastase (0.3 or 0.6 U). Twenty days afterward, mechanical ventilation data were col...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2014.01.009

    authors: Santos LM,de Brito Cervilha DA,Cabral LD,Garcia ÉK,Teixeira VP,Brito JM,Moriya HT,Soncini R

    更新日期:2014-04-01 00:00:00

  • Whole blood hypoxia-related gene expression reveals novel pathways to obstructive sleep apnea in humans.

    abstract::In this study, our goal was to identify the key genes that are associated with obstructive sleep apnea (OSA). Thirty-five volunteers underwent full in-lab polysomnography and, according to the sleep apnea hypopnea index (AHI), were classified into control, mild-to-moderate OSA and severe OSA groups. Severe OSA patient...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2013.08.012

    authors: Perry JC,Guindalini C,Bittencourt L,Garbuio S,Mazzotti DR,Tufik S

    更新日期:2013-12-01 00:00:00

  • Biphasic airway-lung response to anaphylactic shock in Brown Norway rats.

    abstract::Bronchospasm may be part of the response to systemic anaphylaxis in humans. The anaphylactic shock has been characterized in allergic rats, but little data are available on the concurrent changes in airway-lung mechanics. The aim was to describe the respiratory resistance (Rrs) and reactance (Xrs) response to ovalbumi...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2013.07.003

    authors: Barthel G,Zheng F,Demoulin B,Davidson J,Montémont C,Gaburro J,Mertes PM,Marchal F

    更新日期:2013-10-01 00:00:00

  • Effect of domperidone on ventilation and polycythemia after 5 weeks of chronic hypoxia in rats.

    abstract::Chronically hypoxic humans and some mammals have attenuated ventilatory responses, which have been associated with high dopamine level in carotid bodies. Alveolar hypoventilation and blunted ventilatory response have been recognized to be at the basis of Chronic Mountain Sickness by generating arterial hypoxemia and p...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/s1569-9048(03)00065-x

    authors: Gamboa J,Macarlupú JL,Rivera-Chira M,Monge-C C,León-Velarde F

    更新日期:2003-04-15 00:00:00

  • Effect of heavy-intensity 'priming' exercise on oxygen uptake and muscle deoxygenation kinetics during moderate-intensity step-transitions initiated from an elevated work rate.

    abstract::We examined the effect of heavy-intensity 'priming' exercise on the rate of adjustment of pulmonary O2 uptake (τV˙O2p) initiated from elevated intensities. Fourteen men (separated into two groups: τV˙O2p≤25s [Fast] or τV˙O2p>25s [Slow]) completed step-transitions from 20W to 45% lactate threshold (LT; lower-step, LS) ...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2016.09.013

    authors: Nederveen JP,Keir DA,Love LK,Rossiter HB,Kowalchuk JM

    更新日期:2017-01-01 00:00:00

  • Nasal highflow eliminates CO2 from lower airways.

    abstract:INTRODUCTION:Nasal highflow (NHF) has a growing evidence in treatment of hypoxemic respiratory failure. There are preliminary data available about use in acute and chronic type-II-respiratory failure. Unfortunately underlying mechanisms of NHF are not well understood. Increase in airway pressure seems too small to expl...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2017.03.012

    authors: Bräunlich J,Goldner F,Wirtz H

    更新日期:2017-08-01 00:00:00

  • Regulation of alveolar fluid clearance and ENaC expression in lung by exogenous angiotensin II.

    abstract::Angiotensin II (Ang II) has been demonstrated as a pro-inflammatory effect in acute lung injury, but studies of the effect of Ang II on the formation of pulmonary edema and alveolar filling remains unclear. Therefore, in this study the regulation of alveolar fluid clearance (AFC) and the expression of epithelial sodiu...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2011.11.009

    authors: Deng J,Wang DX,Deng W,Li CY,Tong J,Ma H

    更新日期:2012-04-15 00:00:00

  • Respiratory dynamics in phonation and breathing-A real-time MRI study.

    abstract::The respiratory system is a central part of voice production, but for phonation neither the underlying functional relations between diaphragm (DPH) and rib cage (RC), nor differences to normal breathing are yet understood. This study aims to compare respiratory dynamics in phonation and breathing via dynamic MRI of th...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2016.11.007

    authors: Traser L,Özen AC,Burk F,Burdumy M,Bock M,Richter B,Echternach M

    更新日期:2017-02-01 00:00:00

  • Nitric oxide inhibitor altitude-dependently elevates pulmonary arterial pressure in high-altitude adapted yaks.

    abstract::We studied the effect of N(w)-nitro-L-arginine (NLA) on yak pulmonary vascular tone in a climatic (hypobaric/hyperbaric adjusted) chamber. Five young male yaks that had been born and reared at an altitude greater than 3800 m a.s.l. were used. After measuring control values, 20 mg/kg of NLA was administered via the jug...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2004.12.002

    authors: Ishizaki T,Koizumi T,Ruan Z,Wang Z,Chen Q,Sakai A

    更新日期:2005-04-15 00:00:00

  • 24-h blood pressure in Space: The dark side of being an astronaut.

    abstract::Inflight 24-h profiles of blood pressure (BP) and heart rate (HR) were recorded in 2 ESA-astronauts by automatic upper arm cuff measurements. In one astronaut this was combined with Portapres continuous finger blood pressure recordings. It was the intention to contrast the latter to 24-h recordings in an earlier Head-...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2009.05.006

    authors: Karemaker JM,Berecki-Gisolf J

    更新日期:2009-10-01 00:00:00

  • Brainstem-mediated sniffing and respiratory modulation during odor stimulation.

    abstract::The trigeminal and olfactory systems interact during sensory processing of odor. Here, we investigate odor-evoked modulations of brainstem respiratory networks in a decerebrated perfused brainstem preparation of rat with intact olfactory bulbs. Intranasal application of non-trigeminal odors (rose) did not evoke respir...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2016.07.008

    authors: Pérez de Los Cobos Pallares F,Bautista TG,Stanić D,Egger V,Dutschmann M

    更新日期:2016-11-01 00:00:00

  • The evolutionary origin of the mammalian diaphragm.

    abstract::The comparatively low compliance of the mammalian lung results in an evolutionary dilemma: the origin and evolution of this bronchoalveolar lung into a high-performance gas-exchange organ results in a high work of breathing that cannot be achieved without the coupled evolution of a muscular diaphragm. However, despite...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章,评审

    doi:10.1016/j.resp.2010.01.004

    authors: Perry SF,Similowski T,Klein W,Codd JR

    更新日期:2010-04-15 00:00:00

  • Allergen challenge during halothane compared to isoflurane anesthesia induces a more potent peripheral lung response.

    abstract::Allergen instillation in anaesthetized vs. awake animals results in increased distribution of allergen in the lung. Halothane is a more potent bronchodilator of the small airways than isoflurane. As small airways contribute to asthma pathogenesis, we questioned whether intranasal challenge under halothane vs. isoflura...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2013.07.005

    authors: Borges MC,Marchica CL,Narayanan V,Ludwig MS

    更新日期:2013-10-01 00:00:00

  • Regulation of adenosine 5'-triphosphate (ATP)-gated P2X(4) receptors on tracheal smooth muscle cells.

    abstract::We examined the effects of extracellular adenosine 5'-triphosphate (ATP) on single airway smooth muscle (ASM) cells from porcine trachea using a patch-clamp technique. ATP induced a sustained inward current. Phospholipase C inhibitor U-73122 failed to inhibit the current, suggesting the involvement of P2X receptor. A ...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2009.02.002

    authors: Nagaoka M,Nara M,Tamada T,Kume H,Oguma T,Kikuchi T,Zaini J,Moriya T,Ichinose M,Tamura G,Hattori T

    更新日期:2009-03-31 00:00:00

  • Salbutamol improves markers of epithelial function in mice with chronic allergic pulmonary inflammation.

    abstract::We investigated the effects of salbutamol on the markers of epithelial function in a murine model of chronic allergic pulmonary inflammation by recording the ciliary beat frequency (CBF) and the transepithelial potential difference (PD) in vivo. Mice were sensitized and received four challenges of ovalbumin (OVA group...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2011.03.016

    authors: Toledo AC,Arantes-Costa FM,Macchione M,Saldiva PH,Negri EM,Lorenzi-Filho G,Martins MA

    更新日期:2011-07-31 00:00:00

  • Neuromuscular adaptations to respiratory muscle inactivity.

    abstract::Cervical spinal cord injury results in significant functional impairment. It is important to understand the neuroplasticity in response to inactivity of respiratory muscles in order to prevent any associated effects that limit functional recovery. Recent studies have examined the mechanisms involved in inactivity-indu...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章,评审

    doi:10.1016/j.resp.2009.09.002

    authors: Mantilla CB,Sieck GC

    更新日期:2009-11-30 00:00:00

  • The influence of pulmonary receptors on respiratory drive in a rabbit model of pulmonary emphysema.

    abstract::We have observed that pulmonary rapidly adapting receptor activity is greater in emphysematous rats than in controls. Pulmonary receptor activity, if modified by lung disease, may produce an inappropriate drive to breathe which may be perceived as dyspnoea. To investigate the efferent (drive) component of this hypothe...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2006.08.001

    authors: Dallak MA,Pirie LJ,Davies A

    更新日期:2007-04-16 00:00:00

  • Respiratory factors do not limit maximal symptom-limited exercise in patients with mild cystic fibrosis lung disease.

    abstract::To evaluate whether respiratory factors limit exercise capacity in patients with mild cystic fibrosis (CF) lung disease (mean FEV(1) = 76 +/- 7.7% predicted) we stressed the respiratory system of seven patients using added dead space (V(D)). Primary outcomes were exercise duration (Ex(dur)) and maximal oxygen uptake (...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2005.08.003

    authors: Dodd JD,Barry SC,Gallagher CG

    更新日期:2006-06-01 00:00:00

  • Vascular KATP channels mitigate severe muscle O2 delivery-utilization mismatch during contractions in chronic heart failure rats.

    abstract::The vascular ATP-sensitive K+ (KATP) channel is a mediator of skeletal muscle microvascular oxygenation (PO2mv) during contractions in health. We tested the hypothesis that KATP channel function is preserved in chronic heart failure (CHF) and therefore its inhibition would reduce PO2mv and exacerbate the time taken to...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2017.01.009

    authors: Holdsworth CT,Ferguson SK,Colburn TD,Fees AJ,Craig JC,Hirai DM,Poole DC,Musch TI

    更新日期:2017-04-01 00:00:00

  • Different respiratory behaviors disclosed by induced bronchoconstriction in mild asthma patients.

    abstract::Respiratory responses to bronchoconstriction in asthma have been partially assessed and their significance is unclear. In 44 mild asthma patients we investigated respiratory responses during increasing levels of methacholine-induced bronchoconstriction. Inspiratory muscle activity, tidal volume, inspiratory and expira...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2013.08.013

    authors: Lavorini F,Magni C,Chellini E,Camiciottoli G,Pistolesi M,Fontana GA

    更新日期:2013-12-01 00:00:00

  • Details of the physiology of the aerodynamic and heat and moisture transfer in the normal nasal cavity.

    abstract::Anatomically accurate 3D models of 10 healthy nasal cavities are developed from computerized tomography (CT) scan images. Considering anatomical and physiological importance of different parts of the nasal cavity, the surface of each nasal passage is divided to eleven anatomical surfaces. Also the coronal cross sectio...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2020.103480

    authors: Hazeri M,Farshidfar Z,Faramarzi M,Sadrizadeh S,Abouali O

    更新日期:2020-09-01 00:00:00

  • Effect of acetazolamide on respiratory muscle fatigue in humans.

    abstract::Previous studies have demonstrated that carbonic anhydrase inhibition with acetazolamide reduces exercise capacity. The mechanism responsible for this early fatigue is unclear, but may be partly mediated by impaired respiratory muscle function. Inspiratory muscle strength and endurance were assessed in seven healthy m...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2012.08.023

    authors: Gonzales JU,Scheuermann BW

    更新日期:2013-01-15 00:00:00

  • Blood flow and O2 extraction as a function of O2 uptake in muscles composed of different fiber types.

    abstract::We examined how the greater vasodilatory capacity of slow--(ST) versus fast-twitch (FT) muscles impacts the relationship between blood flow (Q ) and O2 uptake (VO2) and, consequently, the O2 extraction (a-vO2 diff.)-to-VO2 relationship. Q was measured with radiolabelled microspheres, while VO2 was calculated by the Fi...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2005.11.004

    authors: Ferreira LF,McDonough P,Behnke BJ,Musch TI,Poole DC

    更新日期:2006-10-27 00:00:00

  • Modulation-demodulation hypothesis of periodic breathing in human respiration.

    abstract::Periodic breathing (PB) is a diseased condition of the cardiorespiratory system, and mathematically it is modelled as an oscillation. Modeling approaches replicate periodic oscillation in the minute ventilation due to a higher than normal gain of the feedback signals from the chemoreceptors coupled with a longer than ...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2018.03.005

    authors: Pal T,Dutta PK,Maka S

    更新日期:2018-06-01 00:00:00

  • Remodeling of the airway smooth muscle cell: are we built of glass?

    abstract::Classical understanding of airway lumen narrowing in asthma has held that the isometric force generated by airway smooth muscle (ASM) must be at every instant in a static mechanical equilibrium with the external load against which the muscle has shortened. It has been established recently, however, that this balance o...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章,评审

    doi:10.1016/s1569-9048(03)00141-1

    authors: Fabry B,Fredberg JJ

    更新日期:2003-09-16 00:00:00

  • Alveolar barrier function assessed by hydrophobic and hydrophilic fluorescent solutes in rabbit lung.

    abstract::Loss of alveolar barrier function is important in the development of pulmonary edema, but quantitation of its integrity has been difficult in the intact lung. We report a new non-radioactive method to assess paracellular and transcellular permeability of alveolar barrier in buffer-perfused rabbit lungs. Changes in alv...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/s1569-9048(02)00150-7

    authors: Huang YC,Piantadosi CA

    更新日期:2002-10-23 00:00:00

  • Postnatal development of eupneic ventilation and metabolism in rats chronically exposed to moderate hyperoxia.

    abstract::Newborn rats chronically exposed to moderate hyperoxia (60% O2) exhibit abnormal respiratory control, including decreased eupneic ventilation. To further characterize this plasticity and explore its proximate mechanisms, rats were exposed to either 21% O2 (Control) or 60% O2 (Hyperoxia) from birth until studied at 3-1...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2014.03.010

    authors: Bavis RW,van Heerden ES,Brackett DG,Harmeling LH,Johnson SM,Blegen HJ,Logan S,Nguyen GN,Fallon SC

    更新日期:2014-07-01 00:00:00

  • Absence of mutations in HCRT, HCRTR1 and HCRTR2 in patients with ROHHAD.

    abstract:BACKGROUND AND OBJECTIVES:Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare pediatric disease of unknown cause. Here, in response to a recent case report describing a ROHHAD patient who suffered from secondary narcolepsy confirmed by an absence of hypocre...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2015.11.002

    authors: Barclay SF,Rand CM,Gray PA,Gibson WT,Wilson RJ,Berry-Kravis EM,Ize-Ludlow D,Bech-Hansen NT,Weese-Mayer DE

    更新日期:2016-01-15 00:00:00

  • Is parasternal intercostal EMG an accurate surrogate of respiratory neural drive and biomarker of dyspnea during cycle exercise testing?

    abstract::Recent evidence suggests that surface electromyography of the parasternal intercostals (EMGpara) can be a non-invasive alternative to diaphragmatic EMG (EMGdi) for estimating neural respiratory drive (NRD) during cardiopulmonary exercise testing (CPET). The purpose of this study was to determine if non-respiratory mus...

    journal_title:Respiratory physiology & neurobiology

    pub_type: 杂志文章

    doi:10.1016/j.resp.2017.03.003

    authors: Ramsook AH,Mitchell RA,Bell T,Calli S,Kennedy C,Lehmann J,Thompson M,Puyat JH,Guenette JA

    更新日期:2017-08-01 00:00:00