Abstract：
:Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disorder that results in death due to respiratory failure. Many genetic defects are associated with ALS; one such defect is a mutation in the gene encoding optineurin (OPTN). Using an optineurin null mouse (Optn-/-), we sought to characterize the impact of optineurin deficiency on respiratory neurodegeneration. Respiratory function was assessed at 6 and 12 mo of age using whole body plethysmography at baseline during normoxia (FiO2: 0.21; N2 balance) and during a respiratory challenge with hypoxia and hypercapnia (FiCO2: 0.07, FiO2: 0.10; N2 balance). Histological analyses to assess motor neuron viability and respiratory nerve integrity were performed in the medulla, cervical spinal cord, hypoglossal nerve, and phrenic nerve. Minute ventilation, peak inspiratory flow, and peak expiratory flow are significantly reduced during a respiratory challenge in 6 mo Optn-/-mice. By 12 mo, tidal volume is also significantly reduced in Optn-/- mice. Furthermore, 12mo Optn-/- mice exhibit hypoglossal motor neuron loss, phrenic and hypoglossal dysmyelination, and accumulated mitochondria in the hypoglossal nerve axons. Overall, these data indicate that Optn-/- mice display neurodegenerative respiratory dysfunction and are a useful model to study the impact of novel therapies on respiratory function for optineurin-deficient ALS patients.
journal_name
Respir Physiol Neurobioljournal_title
Respiratory physiology & neurobiologyauthors
McCall AL,Dhindsa JS,Pucci LA,Kahn AF,Fusco AF,Biswas DD,Strickland LM,Tseng HC,ElMallah MKdoi
10.1016/j.resp.2020.103525subject
Has Abstractpub_date
2020-11-01 00:00:00pages
103525eissn
1569-9048issn
1878-1519pii
S1569-9048(20)30183-Xjournal_volume
282pub_type
杂志文章abstract：:Phrenic motoneurons are located in the cervical spinal cord and innervate the diaphragm muscle, the main inspiratory muscle in mammals. Similar to other skeletal muscles, phrenic motoneurons and diaphragm muscle fibers form motor units which are the final element of neuromotor control. In addition to their role in sus...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2011.06.028
更新日期：2011-10-15 00:00:00
abstract：:Evidence supports the importance of ovarian hormones as potential tools against sleep apneas in women. On one hand, progesterone is largely acknowledged as being a respiratory stimulant that reduces the frequency of apneas, but the underlying mechanisms remain poorly understood. Recent studies in mice showed that the ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2017.01.013
更新日期：2017-05-01 00:00:00
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
更新日期：2017-08-01 00:00:00
abstract：:Hyperventilation is one way to cause activation on the electroencephalogram (EEG) to diagnose brain disorders. The hyperventilation is also known to affect on the delta power in EEG. This study divided the total delta wave into low, middle, and high bands corresponding to the wave frequency. The power in these three d...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2012.02.010
更新日期：2012-04-30 00:00:00
abstract：:The acid-base balance of arterial blood is important for the clinical management of seriously ill patients, especially patients with acute lung injury or acute respiratory distress syndrome. We developed a novel fluorosensor for continuous blood pH monitoring and evaluated its performance both in vitro and in vivo in ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2011.03.009
更新日期：2011-07-31 00:00:00
abstract：:The relationship among airway responsiveness, inflammation and remodelling in asthma is incompletely understood. To investigate potential mechanistic associations, allergen-induced asthma was studied in C57Bl/6 mice. Mice were sensitized and challenged with ovalbumin (OVA) using sub-acute (SA) or chronic (C) protocols...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2009.08.005
更新日期：2009-10-31 00:00:00
abstract：:We hypothesized that progesterone may enhance the hypoxic ventilatory response and reduce the occurrence of apneas in newborn male rats. We studied 10-day-old rats chronically exposed to progesterone (Prog) or vehicle through the milk of lactating mothers. Respiratory and metabolic recordings were performed using whol...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2006.08.007
更新日期：2007-04-16 00:00:00
abstract：:Inconsistent findings regarding the experimental nasal obstruction on respiratory functions in small animals have been reported. The purpose of this study was to investigate the impact of short term forced oral breathing on respiratory functions as well as the therapeutic implication of esophageal intubation in BALB/c...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2014.09.024
更新日期：2015-01-01 00:00:00
abstract：:Changes in airway temperature can result in respiratory responses such as cough, bronchoconstriction and mucosal secretion after cold exposure and hyperventilation after heat exposure. In the present investigation, we examined the activity of bronchopulmonary receptors in response to activators of thermo-sensitive tra...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2011.05.011
更新日期：2011-08-15 00:00:00
abstract：BACKGROUND:Recently, surface EMG of parasternal intercostal muscle has been incorporated in the "ERS Statement of Respiratory Muscle Testing" as a clinical technique to monitor the neural respiratory drive (NRD). However, the anatomy of the parasternal muscle risks confounding EMG "crosstalk" activity from neighboring ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2020.103572
更新日期：2020-11-05 00:00:00
abstract：:Chemoreception in fish is critical for sensing changes in the chemical composition of the external and internal environments and is often the first step in a cascade of events leading to cardiorespiratory and metabolic adjustments. Of paramount importance is the ability to sense changes in the levels of the three resp...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2015.06.007
更新日期：2016-04-01 00:00:00
abstract：:We used quantitative single photon emission computed tomography to estimate the proportion of the observed redistribution of blood flow and ventilation that is due to lung tissue shift with a change in posture. Seven healthy volunteers were studied awake, breathing spontaneously. Regional blood flow and ventilation we...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2006.11.001
更新日期：2007-06-15 00:00:00
abstract：:Lung ventilation is a mechanical process in which the respiratory muscles are acting in concert to remove air in and out of the lungs. Any alteration in the performance of the respiratory muscle may reduce the effectiveness of ventilation. Thus, early diagnosis of their weakness is vital for treatment and rehabilitati...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2008.04.019
更新日期：2008-11-30 00:00:00
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 la...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2017.11.011
更新日期：2018-01-01 00:00:00
abstract：:Dyspnoea, the perception of an unpleasant and/or uncomfortable sensation of breathlessness, offers several physiological, anatomical and teleological analogies with pain. Pain perception has been shown to exist in the newborn, suggesting that dyspnoea may also occur from birth onwards. The perception of breathlessness...
journal_title：Respiratory physiology & neurobiology
pub_type： 社论,评审
doi：10.1016/j.resp.2008.12.001
更新日期：2009-05-30 00:00:00
abstract：:Increased transdiaphragmatic pressure, reduced muscle blood flow, and increased duty cycle have all been associated with a reduction in the center frequency (CFdi) of the diaphragm's electrical activity (EAdi). However, the specific influence of diaphragm activation on CFdi is unknown. We evaluated whether increased d...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2004.11.006
更新日期：2005-03-01 00:00:00
abstract：:Aquaporins (AQPs) are small, integral membrane proteins that facilitate water transport across cell membranes in response to osmotic gradients. Water transport across epithelia and endothelia in the peripheral lung and airways occurs during airway hydration, alveolar fluid transport and submucosal gland secretion. Sev...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2007.02.012
更新日期：2007-12-15 00:00:00
abstract：:Airway smooth muscle maintains airway tone and may assist in adjusting ventilation distribution within the normal lung. Alterations in the properties or the quantity of ASM are likely responsible for some instances of airways hyperresponsiveness to bronchoconstrictive stimuli that is a characteristic of diseases such ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/s1569-9048(03)00151-4
更新日期：2003-09-16 00:00:00
abstract：:Tyrosine kinase inhibitors (TKI), erlotinib and gefitinib are small molecule inhibitors which are used for the treatment of lung cancer. But, the development of drug resistance has been reported as one of the major setbacks in oncology. This study focused on the mechanisms leading to secondary resistance by assessing ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2014.09.022
更新日期：2015-04-01 00:00:00
abstract：:Intermittent hypobaric hypoxia (IHH) exposure, accompanied or not with active recovery, can help to skeletal muscle repair. However, the erythropoietic response elicited can disturb blood rheology and thus alter the oxygen delivery to tissues. Male Sprague-Dawley rats were studied in two basal states: untrained and tr...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2013.12.011
更新日期：2014-02-01 00:00:00
abstract：:We hypothesized that hypocapnia is responsible for increased expiratory resistance during NREM sleep. Hypocapnia was induced by hypoxic hyperventilation in 21 subjects (aged 29.4 ± 7.8 yrs, 10 women, BMI 24.4 ± 4.3 kg/m(2)). Isocapnic hypoxia was induced in 12 subjects of whom, 6 underwent hypocapnic hypoxia in the sa...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2011.04.004
更新日期：2011-07-31 00:00:00
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
更新日期：2006-10-27 00:00:00
abstract：:Our aim was to determine whether maternal cigarette smoking affects arousal and ventilatory responses to hypoxia in infants. Infants born to non-smoking (NS, n = 15) and smoking mothers (SM, n= 9) were studied at 2-5 weeks, 2-3 and 5-6 months. Ventilatory responses to 15% O(2) were determined preceding arousal. At eac...
journal_title：Respiratory physiology & neurobiology
pub_type： 临床试验,杂志文章
doi：10.1016/j.resp.2004.01.004
更新日期：2004-04-20 00:00:00
abstract：:This project aimed to investigate the direction of artificial sputum movement during mechanical ventilation (MV) and bagging (MH) using a tube model. Three solutions of artificial sputum (ultrasonic gel, viscosity 100, 200 and 300 poise (P)) were prepared. About 1 ml of gel was placed in a glass tube connected to a te...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/s1569-9048(02)00071-x
更新日期：2002-09-04 00:00:00
abstract：:To determine the effects of central chemoreceptor stimulation upon sympathetic modulation while minimizing baroreceptor influences, we performed a single-blind, counter-balanced, placebo-controlled trial of a modified hypercapnic/hyperoxic rebreathe protocol stimulus to activate the central chemoreflex. Muscle sympath...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2008.08.010
更新日期：2008-12-31 00:00:00
abstract：:We investigated the signal transmission pathway by which activation of μ-opioid receptors attenuates acetylcholine (ACh) release in bovine trachealis. Electrical stimulation (ES)-induced [(3)H]-ACh release was determined in bovine tracheal smooth muscle strips pre-incubated with either the Gi-protein inhibitor pertuss...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2013.07.021
更新日期：2013-10-01 00:00:00
abstract：:It had been demonstrated previously that the circadian patterns of activity and state of arousal are not essential for the manifestation of the daily patterns of pulmonary ventilation (V(E)), tidal volume (V(T)) and breathing frequency (f). In this study we investigated the extent of the linkage between the circadian ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2006.05.007
更新日期：2007-02-15 00:00:00
abstract：:The role of TRP channels in the ventilatory response to CO2 was investigated in vivo. To this end, the respiration of unrestrained adult TRPM8-, TRPV1- and TRPV4-channel knockout mice was measured using whole-body plethysmography. Under control conditions and hyperoxic hypercapnia, no difference in respiratory paramet...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2019.03.002
更新日期：2019-05-01 00:00:00
abstract：:We have previously shown that mice with near-complete absence of 5-HT neurons (Lmx1bf/f/p) display a blunted hypercapnic ventilatory response (HCVR) and impaired cold-induced thermogenesis, but have normal baseline ventilation (), core body temperature (TCore) and hypoxic ventilatory responses (HVR) at warm ambient te...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2008.08.003
更新日期：2008-12-31 00:00:00
abstract：:The present studies evaluate whether the vagus nerves link the lungs' immune and neural systems by transmitting information through pulmonary nociceptors. Single unit activities from pulmonary nociceptors [C fiber receptors (CFRs) and high threshold Adelta fiber receptors (HTARs)] were recorded from the cervical vagus...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2006.11.005
更新日期：2007-05-14 00:00:00
