Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice.

The most common inherited [correct] form of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting adult motor neurons, is caused by dominant mutations in the ubiquitously expressed Cu-Zn superoxide dismutase (SOD1). In chimeric mice that are mixtures of normal and SOD1 mutant-expressing cells, toxicity to motor neurons is shown to require damage from mutant SOD1 acting within nonneuronal cells. Normal motor neurons in SOD1 mutant chimeras develop aspects of ALS pathology.

Reduction of chemokine IL-8 and RANTES expression in human bronchial epithelial cells by a sea-water derived saline through inhibited nuclear factor-kappaB activation.

The NaCl content of airway surface fluid is believed to be of central importance in lung pathology. To test whether the Na+ concentration could influence the inflammatory response in human bronchial epithelial cells (BECs), we investigated the interleukin (IL)-8 and RANTES expression in BECs exposed to an isotonic sea-water derived low Na+ (ISW) saline compared to isotonic 0.9% NaCl saline.

[Follow-up of a cohort of 2604 colorectal adenomas treated in 1991 and 1992. Search for parameters related to adenomatous recurrence].

Aim: To evaluate the recurrence of colorectal neoplasia after endoscopic resection of adenomas.

Methods: The establishment of a register of colorectal cancers and pre-cancerous lesions for Loire-Atlantique (a French administrative division) led to the recording of files for all subjects with colorectal adenomas. The files for the cohort followed up for the years 1991 and 1992 were re-examined at the end of 1998 to determine the risk factors for recurrence.

Increased seizure susceptibility and proconvulsant activity of anandamide in mice lacking fatty acid amide hydrolase.

A number of recent in vitro studies have described a role for endogenous cannabinoids ("endocannabinoids") as transsynaptic modulators of neuronal activity in the hippocampus and other brain regions. However, the impact that endocannabinoid signals may have on activity-dependent neural events in vivo remains mostly unknown and technically challenging to address because of the short half-life of these chemical messengers in the brain. Mice lacking the enzyme fatty acid amide hydrolase [FAAH (-/-) mice] are severely impaired in their ability to degrade the endocannabinoid anandamide and therefore represent a unique animal model in which to examine the function of this signaling lipid in vivo.

[Diseases of the pulmonary lymphatic system in children].

Diseases of the lymphatic system in children include a group of exceptional conditions difficult to manage. The anatomy of lymphatic system is complex in the lung. Variable from one subject to another, its complex physiology plays an important role in air-blood exchanges occurring in the lung.

[Protein repair therapy in cystic fibrosis].

In the past few years since the cloning of the gene defective in cystic fibrosis, much has been learned on the function of the protein cystic fibrosis transmembrane conductance regulator (CFTR) and on the mechanisms regulating its expression. Based on the current understanding of the processes involved in lung disease progression, a number of approaches have been developed using not only gene therapy, but also pharmacological agents. Several of these agents have been reported to restore function to CFTR with specific mutations.

Spatial and temporal variability of foliar mineral concentration in beech (Fagus sylvatica) stands in northeastern France.

Foliar mineral concentration may provide a basis for monitoring the consequences of long-term environmental changes, such as eutrophication and acidification of soils, or increase in atmospheric CO(2) concentration. However, analytical drifts and inter-tree and year-to-year variations may confound environmental effects on long-term changes in foliar mineral concentration. We have characterized the relative effects of these potentially confounding factors on foliar carbon, nitrogen, phosphorus, calcium, potassium, magnesium and manganese concentrations in 118 pure beech (Fagus sylvatica L.

Time and number discrimination in a bisection task with a sequence of stimuli: a developmental approach.

Children, aged 5 and 8 years, and adults were tested in a bisection task with a sequence of stimuli in which time and number co-varied. In a counting and a non-counting condition, they were instructed either to process the duration of this sequence while ignoring the number of stimuli (temporal bisection), or to process the number of stimuli while ignoring the duration (numerical bisection). In the temporal bisection task, number interfered with the 5-year-olds' temporal performance, indicating that young children did not process time and number independently in a sequence of stimuli when they had to attend to duration.

Distinct cytokine production by lung and blood neutrophils from children with cystic fibrosis.

Inflammation plays a critical role in lung disease progression in cystic fibrosis (CF). This inflammatory process is dominated by a neutrophil influx in the airways. To determine whether the accumulation of neutrophils in the airways of CF patients is associated with an altered function, we analyzed the capacity of neutrophils isolated from the lung compartment and the blood to release the major neutrophil pro- and anti-inflammatory cytokines IL-8 and IL-1-receptor antagonist (ra) spontaneously and in the presence of LPS.

Dexamethasone potentiates keratinocyte growth factor-stimulated SP-A and SP-B gene expression in alveolar epithelial cells.

Keratinocyte growth factor (KGF, or fibroblast growth factor 7) was previously reported to enhance the synthesis of surfactant in alveolar type II cells. We investigated the possible interactions between KGF and a glucocorticoid, dexamethasone (Dex), on surfactant protein (SP) gene expression. In cultured fetal rat type II cells, KGF and Dex induced greater-than-additive stimulating effects on SP-A and SP-B expressions that were enhanced three-fold and 30-fold, respectively, but had only additive effects on SP-C expression.

Converting nonhuman primate dendritic cells into potent antigen-specific cellular immunosuppressants by genetic modification.

T cell depletion plus donor bone marrow cell (BMC) infusion induces long-term kidney allograft survival in a limited number of rhesus macaque recipients. Therefore, there is a need to enhance the tolerogenic activity of donor BMCs. The tolerogenic effect of donor BMCs is ascribed to a veto activity, mediated by a CD8+ subset that upregulates immunoregulatory effector molecules, transforming growth factor-beta1 (TGF-beta), and FasL, after interaction with donor-reactive cytotoxic T lymphocyte precursors (CTLp), leading to clonal inactivation/deletion of donor-reactive CTLp.

Liver disease in pediatric patients with cystic fibrosis is associated with glutathione S-transferase P1 polymorphism.

Liver disease in patients with cystic fibrosis (CF) is inconstant and has not yet been clearly related to any specific risk factor. While the expression of cystic fibrosis transmembrane conductance regulator (CFTR) is restricted to the biliary epithelium in the liver, recent findings indicate that CFTR modulates reduced glutathione (GSH) transport and that CFTR dysfunction creates an imbalance in the antioxidant defense. Among liver detoxifying enzymes, the glutathione S-transferases (GSTs) play a key role in the protection against oxidative stress.

Purification and Characterization of Phosphoribosylpyrophosphate Synthetase from Rubber Tree Latex.

Phosphoribosylpyrophosphate synthetase (PRS; EC 2.7.6.

Cyanobacteria-mediated internal eutrophication in shallow Lake Balaton after load reduction.

Nutrient loads to large, shallow Lake Balaton have been reduced by 45-50% since mid-1980s. While a delayed, but still surprisingly fast recovery was observed in the hypertrophic western areas of the lake, eutrophication followed sewage diversion from the mesotrophic northern basins. We assessed factors that could lead to this unusual response.

Changes in pulmonary mechanics with increasing disease severity in children and young adults with cystic fibrosis.

As forced expiratory volume in 1 second (FEV(1)) is a major predictor of outcome in patients with cystic fibrosis (CF), we investigated the effect of FEV(1) on pulmonary mechanics in children and young adults with CF. We measured respiratory rate; tidal volume; minute ventilation; arterial blood gases; sniff esophageal pressure; dynamic lung compliance; total pulmonary resistance; intrinsic positive end expiratory pressure; and total, elastic, and resistive work of breathing in 32 patients (FEV(1) range: 12-49% predicted). We observed correlations between FEV(1) and Pa(O(2)) (r = 0.

Protective role of retinoic acid from antiproliferative action of TNF-alpha on lung epithelial cells.

Tumor necrosis factor (TNF)-alpha is a key molecule in lung inflammation. We have established the insulin-like growth factor binding protein 2 (IGFBP-2) as a marker associated with the growth arrest of lung alveolar epithelial cells (AEC). Here, we studied the effects of TNF-alpha on AEC proliferation and the putative protective role of retinoic acid (RA).

Depression of diaphragm contractility by nitrous oxide in humans.

Unlabelled: Nitrous oxide is widely used in anesthesia and critical care medicine. The effect of nitrous oxide on diaphragm contractility in humans is unknown. We evaluated the effect of a 50% nitrous oxide-50% oxygen mixture on diaphragm contractility in healthy adult volunteers.

Impairment of rat postnatal lung alveolar development by glucocorticoids: involvement of the p21CIP1 and p27KIP1 cyclin-dependent kinase inhibitors.

It has been shown that glucocorticoids accelerate lung development by limiting alveolar formation resulting from a premature maturation of the alveolar septa. Based on these data, the aim of the present work was to analyze the influence of dexamethasone on cell cycle control mechanisms during postnatal lung development. Cell proliferation is regulated by a network of signaling pathways that converge to the key regulator of cell cycle machinery: the cyclin-dependent kinase (CDK) system.

Role of cyclins in epithelial response to oxidants.

Oxidants are involved in a large variety of pulmonary diseases. Among the various cell types that compose the respiratory system, the epithelial cells appear to be a major target for oxidative stress. When cells are exposed to DNA-damaging agents such as oxidants, a feedback control is activated that acts as a brake on the cell cycle to inhibit entry into the S phase until DNA repair is completed.

Chronic stridor caused by laryngomalacia in children: work of breathing and effects of noninvasive ventilatory assistance.

Breathing pattern, gas exchange, and respiratory effort were assessed in five awake children with chronic stridor caused by laryngomalacia during spontaneous breathing (SB) and noninvasive mechanical ventilation (NIMV). During SB, the youngest children were able to maintain normal gas exchange at the expense of an increased work of breathing as assessed by calculated diaphragmatic pressure-time product (PTPdi), whereas the opposite was observed in the older children. NIMV increased tidal volume, from 8.

In vivo physiologic comparison of two ventilators used for domiciliary ventilation in children with cystic fibrosis.

Objective: Home noninvasive mechanical ventilation (NIMV) is used with increasing frequency for the treatment of patients with respiratory failure caused by cystic fibrosis, yet the optimal mode of ventilation in such children is unknown. We compared the physiologic short-term effects of two ventilators with different modes (one pressure support and the other assist control/volume-targeted [AC/VT]) commonly used for domiciliary ventilation.

Design: Prospective, randomized, crossover comparison of two ventilators with different modes.

Abnormal central complex is a marker of severity in the presence of partial ciliary defect.

Background: Ciliary ultrastructural defects with total lack of dynein arms (DA) cause abnormal mucociliary function leading to the chronic infections observed in primary ciliary dyskinesia. The role of partial ciliary ultrastructural defects, especially those involving the central complex, and their relationship with respiratory symptoms have been less thoroughly investigated.

Objective: In a pediatric population with partial ciliary defects, we determined the relationship(s) between ultrastructural findings, ciliary motility, and clinical and functional features, and evaluated the outcome of this population.