Abstract：
:Amyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disease. ALS selectively causes degeneration in upper and lower (spinal) motor neurons, leading to muscle weakness, paralysis and death by ventilatory failure. Although ventilatory failure is generally the cause of death in ALS, little is known concerning the impact of this disorder on respiratory motor neurons, the consequences of respiratory motor neuron cell death, or the ability of the respiratory control system to "fight back" via mechanisms of compensatory respiratory plasticity. Here we review known effects of ALS on breathing, including possible effects on rhythm generation, respiratory motor neurons, and their target organs: the respiratory muscles. We consider evidence for spontaneous compensatory plasticity, preserving breathing well into disease progression despite dramatic loss of spinal respiratory motor neurons. Finally, we review current and potential therapeutic approaches directed toward preserving the capacity to breathe in ALS patients.
journal_name
Respir Physiol Neurobioljournal_title
Respiratory physiology & neurobiologyauthors
Nichols NL,Van Dyke J,Nashold L,Satriotomo I,Suzuki M,Mitchell GSdoi
10.1016/j.resp.2013.05.016subject
Has Abstractpub_date
2013-11-01 00:00:00pages
429-37issue
2eissn
1569-9048issn
1878-1519pii
S1569-9048(13)00156-0journal_volume
189pub_type
杂志文章,评审abstract：:In order to determine the role of Kir2.2 in the hypercapnic ventilatory response (HCVR) during postnatal development, we measured the response of the Kir2.2-knockout (Kir2.2-/-) mouse in an unanesthetized unrestrained state by means of pressure plethysmography on postnatal days 9-10, 14-15 and 18, and compared the res...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2004.11.003
更新日期：2005-02-15 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：BACKGROUND:A sustained elevation in respiratory drive following removal of the inducing stimulus is known as respiratory long-term facilitation (rLTF). We investigated whether an acute exposure to intermittent negative airway pressure (INAP) elicits rLTF in humans. METHOD:13 healthy males (20.9 ± 2.8 years) undertook ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2019.05.016
更新日期：2019-09-01 00:00:00
abstract：:Previously we have reported that association of cigarette smoke (CS) and chronic hypoxia (CH) interact positively to physiopathologically remodel pulmonary circulation. In present study we have exposed guinea pigs to CS smoke (four cigarettes/day; 3 months; CS) and to chronic hypoxia (12% O(2), 15 days; CH) alone or i...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2011.09.013
更新日期：2011-12-15 00:00:00
abstract：:Genetic deficiency of the muscle chloride channel CLC-1 leads to myotonia congenita in humans as well as myotonia in mice and goats. The hallmark of myotonia is delayed muscle relaxation due to persistent electrical discharges in the muscle. The present study tested the hypothesis that performance of CLC-1 deficient d...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2006.07.003
更新日期：2007-03-15 00:00:00
abstract：:Lung ultrasonography (LUS) and computed tomography (CT) were compared for quantitative assessment of extravascular lung water (EVLW) in 10 isolated bovine lung lobes. LUS and CT were obtained at different inflation pressures before and after instillation with known amounts of hypotonic saline. A video-based quantitati...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2013.04.002
更新日期：2013-07-01 00:00:00
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
更新日期：2013-01-15 00:00:00
abstract：:Obstructive sleep apnea (OSA) is a complex disease in which humans face episodes of intermittent hypoxia and it affects men and women. Patients with OSA present hypertension and sympathetic overactivity among several other dysfunctions. Therefore, one important question remains: are the autonomic dysfunctions associat...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2017.09.003
更新日期：2018-10-01 00:00:00
abstract：:We studied the influence of hypercapnic acidosis and hypoxia on the neural drive to abdominal muscles in anesthetized and decerebrate rats; this information is unavailable despite widespread use of the rat as an experimental model in respiratory physiology and neurobiology. To minimize confounding influences from rece...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2007.01.004
更新日期：2007-08-01 00:00:00
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
更新日期：2018-06-01 00:00:00
abstract：:Maximal inspiratory mouth pressure (MIP) is a common measurement of inspiratory muscle strength, which is often used in a variety of exercises to evaluate the effects of inspiratory muscle training. An understanding of elite athletes' MIP characteristics is needed to guide sport-specific inspiratory muscle training pr...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2016.05.004
更新日期：2016-08-01 00:00:00
abstract：:The adult elephant is unique among mammals in that the pleural membranes are thickened and the pleural cavity is obliterated by connective tissue. It has been suggested that this peculiar anatomy developed because the animal can snorkel at depth, and this behavior subjects the microvessels in the parietal pleura to a ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/s1569-9048(03)00199-x
更新日期：2003-11-14 00:00:00
abstract：:This study tested the hypothesis that, by increasing the volume available for tidal expansion (inspiratory capacity, IC), bi-level positive airway pressure (BiPAP™) would lead to greater beneficial effects on dyspnea and exercise intolerance in comorbid heart failure (HF)-chronic obstructive pulmonary disease (COPD) t...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2019.04.008
更新日期：2019-08-01 00:00:00
abstract：:It has been shown that tiny gas nanobubbles form spontaneously on a smooth hydrophobic surface submerged in water. These nanobubbles were shown to be the source of gas micronuclei from which bubbles evolved during decompression of silicon wafers. We suggest that the hydrophobic inner surface of blood vessels may be a ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2013.04.013
更新日期：2013-08-01 00:00:00
abstract：:Many lung and central nervous system disorders require robust and appropriate physiological responses to assure adequate breathing. Factors undermining the efficacy of ventilatory control will diminish the ability to compensate for pathology, threatening life itself. Although most of these same disorders are associate...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2011.06.017
更新日期：2011-09-30 00:00:00
abstract：:In order to examine, whether the lobeline-induced cough is a true reflex or a voluntary effort to get rid of its irritating sensations in the upper respiratory tract, we systematically studied the cough response to lobeline, of subjects who were unable to make conscious discriminations i.e. were either comatose (n=4) ...
journal_title：Respiratory physiology & neurobiology
pub_type： 临床试验,杂志文章
doi：10.1016/j.resp.2004.09.001
更新日期：2005-01-15 00:00:00
abstract：:We investigated the influence of airway obstruction in the complexity of the airflow pattern in COPD and its use as a marker of disease activity. The sample entropy (SampEnV') and the variability (SDV') of the airflow pattern were measured in a group of 88 subjects with various levels of airway obstruction. Airway obs...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2013.12.004
更新日期：2014-02-01 00:00:00
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
更新日期：2009-03-31 00:00:00
abstract：:The mechanisms of cough in asthma are unclear. Asthma is associated with an oxidative stress. Many reactive oxygen species sensitize or activate sensory C-fibers which are capable to induce cough. It was hypothesized that oxidative stress in the airways might contribute to the cough severity in asthma. Exhaled breath ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2012.04.006
更新日期：2012-05-31 00:00:00
abstract：AIMS:Quantitatively measure and validate analysis of neural respiratory drive (NRD) using a commercial polysomnography system in children during sleep. METHOD:Surface electromyogram of the diaphragm (sEMGdi) recorded from primary snoring children were analysed. A subset was re-analysed to assess intra- and inter-inves...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2017.02.004
更新日期：2017-05-01 00:00:00
abstract：:During prolonged anoxia at low temperature, freshwater turtles develop high plasma concentrations of both lactate and calcium. At these concentrations the formation of the complex, calcium lactate, normally of little biological significance because of the low association constant for the reaction, significantly reduce...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2004.06.020
更新日期：2004-12-15 00:00:00
abstract：:Breathing movements require the coordinated recruitment of cranial and spinal motoneurons innervating muscles of the upper airway and ribcage. A significant part of respiratory motoneuron development and maturation occurs prenatally to support the generation of fetal breathing movements in utero and sustained breathin...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章,评审
doi：10.1016/j.resp.2005.03.017
更新日期：2005-11-15 00:00:00
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
更新日期：2011-07-31 00:00:00
abstract：OBJECTIVE:Consequences of OSAS include excessive daytime sleepiness, divided sleep architecture, impaired neurocognitive performance, and significant psychosocial disruption. In this study we aimed to evaluate sleep spindles changes before and after PAP treatment in patient with OSA. METHODS:Seventy-three consecutive ...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2017.09.008
更新日期：2018-01-01 00:00:00
abstract：:We previously found that maternal cigarette smoke (CS) exposure resulted in impairment of central chemoreception and oxidative stress and mitochondrial dysfunction of parafacial respiratory group (pFRG, a critical site for mammalian central chemoreception) in neonatal rats. The present work was carried out to identify...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2020.103383
更新日期：2020-03-01 00:00:00
abstract：:The regulatory effect of substance P on respiration is mediated via neurokinin (NK) receptors. While previous studies suggest that NK-1 receptors are involved, little is known about the role NK-2 receptors in ventilatory responses to hypoxia. Ventilatory responses to acute hypoxia (8% O2 in N2) were measured by indire...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2007.07.012
更新日期：2007-11-15 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：:If, as postulated, the end of the steady state phase (φ2) of cardiovascular responses to apnoea corresponds to the physiological breaking point, then we may hypothesize that φ2 should become visible if exercise apnoeas are performed in pure oxygen. We tested this hypothesis on 9 professional divers by means of continu...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2018.05.003
更新日期：2018-09-01 00:00:00
abstract：:The physiologic function of nasal nitric oxide (NO) release is unknown. In prior experiments, topical NG-nitro-L-arginine methyl ester (L-NAME) on nasal mucosa reduced exhaled nasal NO output and caused daytime sleepiness. We hypothesized that nasal NO output is reduced at night during the sleep period. We measured ex...
journal_title：Respiratory physiology & neurobiology
pub_type： 杂志文章
doi：10.1016/j.resp.2006.08.002
更新日期：2007-04-16 00:00:00
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
更新日期：2005-04-15 00:00:00
