Abstract:
:Rett syndrome is a neurodevelopmental disease due to Mecp2 gene mutations that is associated to complex neurological symptoms, with bioaminergic deficits and life-threatening apneas related to sudden and unexpected death. In male mice, Mecp2-deficiency similarly induces medullary bioaminergic deficits, severe apneas and short life span. Here, we show that long-term oral treatment of Mecp2-deficient male mice with desipramine, an old drug of clinical use known to block norepinephrine uptake and to strengthen its synaptic effects, significantly alleviates their breathing symptoms and prolongs their life span. Although these mouse results identify desipramine as the first oral pharmacological treatment potentially able to alleviate breathing symptoms of Rett syndrome, we recommend further studies of desipramine effects in Mecp2-deficient mice before attempting any clinical trials in Rett patients.
journal_name
Respir Physiol Neurobioljournal_title
Respiratory physiology & neurobiologyauthors
Zanella S,Mebarek S,Lajard AM,Picard N,Dutschmann M,Hilaire Gdoi
10.1016/j.resp.2007.08.009subject
Has Abstractpub_date
2008-01-01 00:00:00pages
116-21issue
1eissn
1569-9048issn
1878-1519pii
S1569-9048(07)00222-4journal_volume
160pub_type
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journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2010.06.004
更新日期:2010-08-31 00:00:00
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更新日期:2020-03-01 00:00:00
abstract::In animals, high-intensity unilateral stimulation of the phrenic nerve results in short-latency inhibition of phrenic and intercostal nerve activity bilaterally. This study provides the first demonstration in human subjects of a short-latency inhibitory response in the contralateral scalene, parasternal intercostal an...
journal_title:Respiratory physiology & neurobiology
pub_type: 临床试验,杂志文章
doi:10.1016/s1569-9048(03)00161-7
更新日期:2003-10-16 00:00:00
abstract::This article constitutes a review of the studies performed by the group of the late A. Guz and other authors on the subjects of lung reflexes and perceptions of respiration. The experimental data suggest that the lung inflation and deflation reflexes are present in man, mediated by large myelinated afferent nerve fibr...
journal_title:Respiratory physiology & neurobiology
pub_type: 历史文章,杂志文章,评审
doi:10.1016/j.resp.2015.06.005
更新日期:2015-10-01 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::Recently, we have shown that the expired CO2 gas volume versus tidal volume (VCO2-VT) curve is a useful tool for assessing unevenness of ventilation because it allows the separation of tidal volume into three functional compartments: (a) the CO2-free expired air (V0), (b) the transitional volume (Vtr), (c) the alveola...
journal_title:Respiratory physiology & neurobiology
pub_type: 临床试验,杂志文章
doi:10.1016/j.resp.2004.01.005
更新日期:2004-06-25 00:00:00
abstract::Respiratory responses to single intermittent hypoxia (5 min 21% O(2), 5 min 8% O(2) X6) in 5-6, 10-11, 21-22 and 26-27 day-old piglets, and to recurrent six daily intermittent hypoxia in 10-11 and 26-27 day-old piglets were assessed. Substance P binding in the piglets' brainstem immediately after the last hypoxic epis...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2004.07.013
更新日期:2004-10-12 00:00:00
abstract::To assess the occurrence of tidal expiratory flow limitation (EFL) and/or dynamic pulmonary hyperinflation (DH) in chronic heart failure (CHF) during exercise 15 patients with stable systolic CHF, aged 69 ± 6yr, underwent pulmonary function testing and incremental cardio-pulmonary exercise testing. They subsequently p...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2013.06.017
更新日期:2013-10-01 00:00:00
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journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2011.02.001
更新日期:2011-05-31 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 dependence of metabolic rate (MR) on body mass (M) is described by the general allometric equation MR=aM(b), where, a is a proportionality coefficient and b is the mass exponent. Darveau et al. [Nature 417 (2002), 166] proposed a novel 'multiple-causes' allometric cascade model as a unifying principle of the scali...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/s1569-9048(03)00027-2
更新日期:2003-04-15 00:00:00
abstract::It is well established that women exhibit several anatomic and physiologic characteristics that distinguish their responses to exercise from those of men. These factors have been shown to influence the training response and contribute to lower maximal aerobic power in women. Additionally, the reproductive hormones, es...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章,评审
doi:10.1016/j.resp.2005.10.010
更新日期:2006-04-28 00:00:00
abstract::At high altitude, hypoxia amplifies oxyhemoglobin saturation (SPO2) swings with changes in respiratory mechanics. Our objective was to examine the effects of posture on SPO2 and determine predictors of postural SPO2 changes in highlanders. 50 native highlanders from Puno, Peru (3825 m) assumed supine and upright-seate...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2019.04.015
更新日期:2019-08-01 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::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::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 kn...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章,评审
doi:10.1016/j.resp.2013.05.016
更新日期:2013-11-01 00:00:00
abstract::The aim of this study was to compare the ventilatory responses of C57BL6 female and male mice during a 15 min exposure to a hypoxic-hypercapnic (H-H) or a hypoxic (10% O(2), 90% N(2)) challenge and subsequent return to room air. The ventilatory responses to H-H were similar in males and females whereas there were pron...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2012.11.010
更新日期:2013-02-01 00:00:00
abstract::Effect of time and phosphatidylcholines (PCs) on lubrication of damaged mesothelium has been investigated. Marked increase in coefficient of kinetic friction (μ) of pleural specimens after mesothelial blotting and rewetting decreased by 23.4±3.5%, 41.8±3.8%, and 40.5±2.7% after 30min, 1h, and 2h. Hence, damaged mesoth...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2014.08.002
更新日期:2014-11-01 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::Functional genomics is a systematic and high-throughput effort to analyze the functions of genes and gene products. Functional genomics is divided into gene- and phenotype-driven approaches. Gene-driven approaches to the functional genomics of sleep have demonstrated that transcripts of many genes change as a function...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章,评审
doi:10.1016/s1569-9048(03)00045-4
更新日期:2003-05-30 00:00:00
abstract::Recent in vitro data suggest that astrocytes may modulate respiration. To examine this question in vivo, we treated 5-day-old rat pups with methionine sulfoximine (MS), a compound that alters carbohydrate and glutamate metabolism in astrocytes, but not neurons. MS-treated pups displayed a reduced breathing frequency (...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2005.01.009
更新日期:2005-05-12 00:00:00
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
更新日期:2006-06-01 00:00:00
abstract::Peroxisome proliferator-activated receptor (PPAR)-β/δ is a transcription factor that belongs to the PPAR family, but the role of PPAR-β/δ in acute lung injury (ALI) induced by hyperbaric oxygen is unknown. In this study we investigated if PPAR-β/δ activation protects from hyperoxia-induced ALI in a rat model. ALI was ...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2014.04.004
更新日期:2014-08-01 00:00:00
abstract::Computational model analysis has been used widely to understand and interpret complexity of interactions in the pulmonary system. Pulmonary blood transport is a multi-scale phenomenon that involves scale-dependent structure and function, therefore requiring different model assumptions for the microcirculation and the ...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章,评审
doi:10.1016/j.resp.2008.02.016
更新日期:2008-11-30 00:00:00
abstract::Submaximal exercise diaphragm blood flow (BF) is elevated in young chronic heart failure (CHF) rats, while it is unknown if this occurs in older animals. Respiratory and hindlimb muscle BFs (radiolabeled microspheres) were measured at rest and during submaximal exercise (20m/min, 5% grade) in older healthy (n=7) and C...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2017.05.002
更新日期:2017-09-01 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::Recently, we reported that dyspnea on exertion is strongly associated with an increased oxygen cost of breathing in otherwise healthy obese women; the mechanism of dyspnea on exertion in obese men is unknown. Obese men underwent measurements of body composition, fat distribution, pulmonary function, steady state and m...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2012.10.007
更新日期:2013-01-15 00:00:00
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
更新日期:2017-01-01 00:00:00
abstract::The resurgence of pertussis has been the subject of considerable debate. Hypotheses to explain increased reporting in developed countries have focused mainly on three aspects: (1) increased recognition of the disease in adolescents and adults; (2) waning of vaccine-induced immunity and (3) loss of vaccine efficacy due...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章,评审
doi:10.1016/j.resp.2013.02.007
更新日期:2013-06-01 00:00:00
abstract:BACKGROUND:The impact of aerobic training on pulmonary function by modulating gene expression of estrogen receptor-alpha (ERα), sex hormones and 25-hydroxy vitamin D (Vit D) in postmenopausal women (PMW) with Vit D deficiency is uncertain. OBJECTIVE:The purpose of this study was to examine the effect of 12 weeks of mo...
journal_title:Respiratory physiology & neurobiology
pub_type: 杂志文章
doi:10.1016/j.resp.2020.103510
更新日期:2020-10-01 00:00:00