Abstract:
:The purpose of this investigation was twofold: (1) to identify and characterize the enzymatic ATP hydrolysis system of epithelial cilia, and (2) to develop a quantitative, biochemical test for the ciliotoxic cystic fibrosis (CF) factor based on inhibition of ATP utilization by ciliary preparations. Our rationale for selecting this system for CF factor analysis relates to the tight and essential mechanochemical coupling of functioning cilia. Using rabbit tracheal epithelium as the source, a high molecular weight (greater than 200,000) ATPase was identified, partially purified, and extensively characterized. The properties of this protein were similar to those observed in previous studies of others with flagellar and ciliary dynein (the motility-associated ATPase) isolated from microorganisms. Analysis of the pH profile revealed a broad range of high enzymatic activity between 6.5 and 9. Studies with potential cation activators showed that the enzyme is activated equally by either Ca2+ or Mg2+ in equimolar concentrations. No activation occurred in the presence of Zn2+, Na+, H+, or Na+ plus K+ and the effect of Mg2+ or Ca2+ was not inhibited by Na+, K+, or Na+ plus K+. The enzyme hydrolyzed Mg2+-containing solutions of UTP, CTP, and ADP at 51-54% the rate of ATP dephosphorylation, whereas Mg-deoxy-ATP was hydrolyzed 79% as effectively as ATP. Using a newly devised, analytical technique with [gamma-32P]ATP as the substrate, the ATP hydrolysis of various ciliary preparations from rabbit trachea and oyster gill (including motile suspensions) was monitored in the presence of sera from CF homo- and heterozygotes. Reproducible rates of ATP dephosphorylation averaging 27 nmol/min/mg protein were demonstrable with homogenates of ciliated epithelium. None of the test systems evaluated, however, were capable of demonstrating CF-related differences in ATPase activity or ATP utilization. Although these attemps have been unsuccessful thus far, the approach described in this report provides an example of an objective, quantitative, biochemical assessment of ciliary function.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Farrell PM,Fox GN,Spicer SSdoi
10.1203/00006450-197602000-00012subject
Has Abstractpub_date
1976-02-01 00:00:00pages
127-35issue
2eissn
0031-3998issn
1530-0447journal_volume
10pub_type
杂志文章abstract::To determine whether epidermal growth factor (EGF) or EGF-like transforming growth factors (TGFs) are present in ovine fetal tissues, we have tested acid-ethanol extracts of ovine fetal kidney for the ability to induce anchorage-independent growth of normal rat kidney fibroblasts in soft agar and to compete with 125I-...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198711000-00026
更新日期:1987-11-01 00:00:00
abstract::To clarify the prevalence of Japanese children thought to be at a risk for infection with GB virus-C (GBV-C)/hepatitis G virus (HGV), we investigated the detection rates of serum GBV-C/ HGV ribonucleic acid (RNA) by reverse transcription-seminested PCR and serum anti-HGV-E2 antibody by ELISA in 162 children with histo...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199901000-00021
更新日期:1999-01-01 00:00:00
abstract::Sildenafil (Viagra) has been shown to be an effective pulmonary vasodilator and is increasingly used in patients with pulmonary hypertension. Its effects on the cerebral circulation are unclear and have not yet been described. We investigated the effect of i.v. sildenafil treatment on cerebral oxygenation in 13 childr...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/01.pdr.0000198772.26417.66
更新日期:2006-03-01 00:00:00
abstract::The pathophysiology of kwashiorkor, a severe edematous manifestation of malnutrition, is still poorly understood. The syndrome is, however, known to be associated with alterations in redox metabolism. To further elucidate the role of oxidative stress in kwashiorkor, we carried out a longitudinal study on the major blo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200102000-00018
更新日期:2001-02-01 00:00:00
abstract::In this study we examined the body composition at onset of puberty in intrauterine growth retarded (IUGR), postnatal food restricted (FR), and control male and female rats. IUGR was induced by ligation of the uterine artery on d 17 of gestation and FR by litter enlargement to 20 pups per mother from d 2 after birth un...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200110000-00009
更新日期:2001-10-01 00:00:00
abstract::Platelet volume was measured in 20 healthy children, 18 patients with acute idiopathic thrombocytopenic purpura (ITP), 24 patients with chronic ITP, 22 patients with aplastic anemia, and 17 patients with acute leukemia in childhood. The authors compared the platelet volume of these patients by use of peak platelet vol...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198002000-00013
更新日期:1980-02-01 00:00:00
abstract::Developmental differences in myocardial performance are known to exist. It is likely that the profile of protein isoforms present on the developing thin filament contributes to these observed differences. We have prepared thin filaments from developing and mature rat hearts by using an immunoprecipitation procedure de...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199109000-00006
更新日期:1991-09-01 00:00:00
abstract::Sarcoplasmic reticulum (SR) Ca release has been shown not to be the predominant mechanism responsible for excitation-contraction (E-C) coupling in fetal myocytes. However, most of the studies have been conducted either on primary cultures or acutely isolated cells, in which an apparent reduction of ryanodine receptor ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31820bc69b
更新日期:2011-04-01 00:00:00
abstract::Human milk was fractionated to obtain a partially purified growth factor preparation. The growth factor in this fraction, designated as human milk growth factor III, exhibits chromatographic and biological characteristics similar to epidermal growth factor-urogastrone. Pretreatment of mice with human milk growth facto...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198509000-00009
更新日期:1985-09-01 00:00:00
abstract::Evaluation of nonlinear heart rate (HR) dynamics has received considerable attention in the pediatric literature because such analyses not only provide insight into underlying control mechanisms, but may also help to differentiate between normal and abnormal infants. The purpose of this study was to determine, in eigh...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199807000-00018
更新日期:1998-07-01 00:00:00
abstract::Cerebral concentrations of phenylalanine (PHE) were measured by means of quantitative in vivo 1H MR spectroscopy in 8 adult patients treated early for phenylketonuria type I. A 1.5-Tesla routine magnetic resonance scanner, localization sequence with short echo time (20 ms), and a fully automated data processing scheme...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199511000-00005
更新日期:1995-11-01 00:00:00
abstract::Sequential magnitude and the rate of change in tracheal mechanics after application of intermittent positive pressure (IPP), and the time constant, tau, of this deformation were determined in fetal (21 and 27 days gestation), neonatal (term, 31 days gestation) and adult rabbits. In vitro tracheal mechanics were determ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198210000-00006
更新日期:1982-10-01 00:00:00
abstract::Tolazoline is used in neonatal intensive care to treat hypoxia secondary to persistent pulmonary hypertension of the newborn. Its use is often complicated by systemic hypotension. We compared the effect of tolazoline-induced hypotension on organ blood flow, regional brain blood flow, and cerebral metabolism in hypoxic...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199008000-00007
更新日期:1990-08-01 00:00:00
abstract::Sex steroids accelerate bone maturation, but it is believed that estrogen action is needed for terminal epiphyseal fusion. In this study, we investigated the effects of a new estrogen-blocking agent, Faslodex (ICI 182,780), on estrogen-accelerated skeletal maturation in immature mice. On day-of-life 2 through 8, mice ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199909000-00004
更新日期:1999-09-01 00:00:00
abstract::To describe the relation between scalp blood flow and transcutaneous PO2 and PCO2, these three signals were recorded simultaneously in eight experiments on four healthy lambs in the first weeks after birth. Scalp blood flow was recorded by a laser Doppler flow sensor that was incorporated in the transcutaneous PO2 ele...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199005000-00005
更新日期:1990-05-01 00:00:00
abstract::We determined whether closing the ductus arteriosus of the fetal lamb several d before birth would cause persistent pulmonary hypertension after birth. Six experimental fetuses who had their ductus arteriosus ligated 3-17 d before delivery, three control fetuses who had sham ligation of the ductus arteriosus 14 d befo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198903000-00005
更新日期:1989-03-01 00:00:00
abstract::Treatment with phototherapy or with the lipase inhibitor orlistat decreases plasma unconjugated bilirubin (UCB) concentrations in hyperbilirubinemic Gunn rats. We investigated the mechanism(s) underlying the effects of orlistat, phototherapy, and combined treatment, using steady-state 3H-UCB kinetics. After three week...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000203180.79636.98
更新日期:2006-04-01 00:00:00
abstract::This study was made to determine the life span of adult red cells transfused to early preterm infants. Nineteen very preterm infants (birth weight, 878.7 +/- 221 g; gestational age, 26.8 +/- 1.5 wk at birth) were sampled weekly after their last blood transfusion to determine the level (%) of fetal Hb in their circulat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199707000-00002
更新日期:1997-07-01 00:00:00
abstract:INTRODUCTION:Data regarding neonatal arterial ischemic stroke (NAIS) topography are still sparse and inaccurate. Despite the importance of locating NAIS to predict the long-term outcome of neonates, a map of arterial territories is not yet available. Our aim was therefore to generate the first three-dimensional map of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0724-x
更新日期:2020-06-01 00:00:00
abstract::The use of nebulized racemic epinephrine to improve ventilatory function in the newborn postextubation was studied. Forty-five infants intubated for more than 3 days were randomized to receive racemic epinephrine by nebulization immediately postextubation or to receive only warmed, humidified gas. Measurements of air ...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1203/00006450-198704000-00012
更新日期:1987-04-01 00:00:00
abstract::BackgroundSpecific HLA-DQ genes have been recognized as necessary - but not sufficient - factors for the occurrence of Celiac Disease (CD). Through a meta-analysis, evaluating the distribution of CD-related HLA genotypes in children, we aimed at providing insights for a potential widened screening strategy.MethodsAfte...
journal_title:Pediatric research
pub_type: 杂志文章,meta分析
doi:10.1038/pr.2017.307
更新日期:2018-03-01 00:00:00
abstract::This study characterizes cardiorespiratory dysregulation in young girls with MECP2 mutation-confirmed Rett syndrome (RS). Respiratory inductance plethysmography of chest/abdomen and ECG was obtained during daytime wakefulness in 47 girls with MECP2 mutation-confirmed RS and 47 age-, gender-, and ethnicity-matched cont...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000238302.84552.d0
更新日期:2006-10-01 00:00:00
abstract::Yesterday's immunodeficiencies emphasized the clinical and familial associations of the syndromes and date from the 1920s (ataxia-telangiectasia, chronic mucocutaneous candidiasis), the 1930s (Wiskott-Aldrich syndrome), skipping the 1940s, but blossoming in the 15-y period from 1950 to 1965. In this period, primary im...
journal_title:Pediatric research
pub_type: 历史文章,杂志文章,评审
doi:10.1203/00006450-199305001-00007
更新日期:1993-01-01 00:00:00
abstract::It is increasingly recognized that the abnormal physiologic consequences of pulmonary hypertension (PH) may contribute to poor cardiopulmonary health in premature babies. Conflicting literature has led to clinical uncertainty, pathological misinterpretation, and variability in treatment approaches among practitioners....
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2017.200
更新日期:2017-12-01 00:00:00
abstract::The possible role played by albumin in regulating brain metabolism during development has been studied. The effects of fatty acid-free BSA on lactate, glucose, 3-hydroxybutyrate, and glutamine oxidation and lipogenesis by rat neurons and astrocytes from primary culture were studied. The rate of lactate oxidation and l...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199312000-00002
更新日期:1993-12-01 00:00:00
abstract::Irreversible congenital heart block (CHB) and the transient rash of neonatal lupus are strongly associated with maternal antibodies to SSA/Ro and SSB/La proteins; however, the precise mechanism by which these antibodies mediate organ-specific injury is not yet defined. Culturing of keratinocytes has provided critical ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199902000-00018
更新日期:1999-02-01 00:00:00
abstract::The fetal inflammatory response has been suggested as causal in neonatal morbidity. Serial levels of circulating cytokines were evaluated in 74 infants with a mean gestational age (GA) of 27.1 wk. Pro-inflammatory [tumor necrosis factor-alpha (TNF-alpha), interferon-gamma (IFN-gamma), IL-1 beta, IL-2, IL-6, IL-8, IL-1...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000182592.76702.E8
更新日期:2005-11-01 00:00:00
abstract::Chondro-osseous tissue from five patients with diastropic dwarfism was studied by histologic, histochemical, and electron microscopic methods. The major abnormalities observed were: 1) irregular distribution of chondrocytes undergoing degeneration in the resting cartilage; 2) abnormal distribution of collagens in the ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197908000-00007
更新日期:1979-08-01 00:00:00
abstract::We banded the trachea of 4-wk-old male rats and studied lung growth, lung morphometry, and static pulmonary mechanics 4 wk later. We compared the experimental animals to sham-operated male littermates of the same age, 8-wk-old normal male littermates, and weight-matched rats of a younger age. Tracheal banded animals w...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198103000-00014
更新日期:1981-03-01 00:00:00
abstract::Cell-mediated immunity is not well characterized in very low birth weight infants, and abnormalities may represent a significant vulnerability to infection. This report describes 165 serial studies in 58 infants between 700 and 1300 g birth weight during the first 8 wk of life. Two ml of blood were drawn at 2-wk inter...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198805000-00003
更新日期:1988-05-01 00:00:00