Abstract:
:The secreted protein sonic hedgehog (Shh) is essential for normal development of many organs. Targeted disruption of Shh in mouse leads to near complete absence of craniofacial skeletal elements at birth, and mutation of SHH in human causes holoprosencephaly (HPE), frequently associated with defects of derivatives of pharyngeal arches. To investigate the role of Shh signaling in early pharyngeal arch development, we analyzed Shh mutant embryos using molecular markers and found that the first pharyngeal arch (PA1) was specifically hypoplastic and fused in the midline, and remaining arches were well formed at embryonic day (E) 9.5. Molecular analyses using specific markers suggested that the growth of the maxillary arch and proximal mandibular arch was severely defective in Shh-null PA1, whereas the distal mandibular arch was less affected. TUNEL assay revealed an increase in the number of apoptotic signals in PA1 of Shh mutant embryos. Ectodermal expression of fibroblast growth factor (Fgf)-8, a cell survival factor for pharyngeal arch mesenchyme, was down-regulated in the PA1 of Shh mutants. Consistent with this observation, downstream transcriptional targets of Fgf8 signaling in neural crest-derived mesenchyme, including Barx1, goosecoid, and Dlx2, were also down-regulated in Shh-null PA1. These results demonstrate that epithelial-mesenchymal signaling and transcriptional events coordinated by Shh, partly via Fgf8, is essential for cell survival and tissue outgrowth of the developing PA1.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Yamagishi C,Yamagishi H,Maeda J,Tsuchihashi T,Ivey K,Hu T,Srivastava Ddoi
10.1203/01.pdr.0000199911.17287.3esubject
Has Abstractpub_date
2006-03-01 00:00:00pages
349-54issue
3eissn
0031-3998issn
1530-0447pii
59/3/349journal_volume
59pub_type
杂志文章abstract::Completely penetrant mutations in the surfactant protein B gene (SFTPB) and >75% reduction of SFTPB expression disrupt pulmonary surfactant function and cause neonatal respiratory distress syndrome. To inform studies of genetic regulation of SFTPB expression, we created a catalogue of SFTPB variants by comprehensive r...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3180a03232
更新日期:2007-08-01 00:00:00
abstract:BACKGROUND:Infant adiposity is linked to both high maternal fat mass (FM) and excessive gestational FM gain, whereas the association with maternal adipokines is less clear. The aim was to determine how levels of maternal leptin, the soluble leptin receptor (sOB-R), adiponectin, and FM during pregnancy were linked to in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-01115-9
更新日期:2020-09-14 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::A strongly positive tuberculin skin reaction (> 1.5 cm2) was observed during the acute phase of the illness in 11 children with Kawasaki disease (KD), but not in control pediatric patients with other febrile infections (41 patients) or diseases similar to KD (9 patients). The cutaneous sensitivity to intermediate stre...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199704000-00017
更新日期:1997-04-01 00:00:00
abstract:INTRODUCTION:To examine the immune-modulatory effects of probiotics during early infancy, Bifidobacterium breve M-16V (B. breve) was administered to rat pups during the newborn or weaning period, and the expression of inflammatory genes was investigated using a cDNA microarray and real-time PCR. RESULTS:After B. breve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2011.11
更新日期:2012-01-01 00:00:00
abstract::Surface active aerosols were produced from aqueous dispersions of mixed lipids (CLL), extracted from bovine lung lavage. Particle size distributions were measured as a function of humidity for two types of aerosol generators: ultrasonic and jet. Lipid dispersions before aerosolization were prepared by sonication in an...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198309000-00012
更新日期:1983-09-01 00:00:00
abstract::Chronic fetal anemia causes polyhydramnios and fetal hydrops and is associated with increased fetal diuresis and natriuresis. To determine the role of atrial natriuretic peptide (ANP) in the renal adaptation to chronic fetal anemia we studied the effects of HS-142-1 (HS), a specific inhibitor of the guanylate cyclase-...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199511000-00015
更新日期:1995-11-01 00:00:00
abstract::The placenta is essential to nutrition before birth. Recent work has shown that a range of clearly defined alterations can be found in the placentas of infants with intrauterine growth restriction (IUGR). In the mouse, a placental specific knockout of a single imprinted gene, encoding IGF-2, results in one pattern of ...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/01.PDR.0000181381.82856.23
更新日期:2005-11-01 00:00:00
abstract::Response characteristics of a transcutaneous PO2 electrode to step changes in PaO2 were determined. In vitro lag time and 95% response time of a transcutaneous PO2 monitor were compared to in vivo response. Release of arterial occlusion was used to produce rapid local PaO2 changes in human infants and adults. In vitro...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198102000-00012
更新日期:1981-02-01 00:00:00
abstract::Wu and colleagues analyzed the placental pathology from a subset of the neonates in the NEATO trial who had reports available and correlated the placental pathology findings with outcomes. This study highlights the importance of placental pathology, and its potential to bring precision medicine to critically-ill neona...
journal_title:Pediatric research
pub_type: 评论,杂志文章
doi:10.1038/s41390-020-0807-8
更新日期:2020-04-01 00:00:00
abstract:BACKGROUND:Staphylococcus epidermidis (SE) is an important cause of late-onset sepsis in neonates. SE frequently produces a polysaccharide intercellular adhesin (PIA) biofilm, important in the pathogenesis of these infections. Little is known about how the neonatal innate immune system reacts to SE biofilm-associated i...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2012.193
更新日期:2013-03-01 00:00:00
abstract::We determined the inhibitory effects of concentrations of tin- and zinc protoporphyrin (1-100 microM) and mesoporphyrin (0.1-10 microM) on the in vitro heme oxygenase (HO) (E.C.1.14.99.3) activity in liver, spleen, and intestine 13,000 x g tissue supernatants from fasted adult male Wistar rats through measurement of c...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198910000-00015
更新日期:1989-10-01 00:00:00
abstract::Serial plasma concentrations of immunoreactive 6-keto-prostaglandin F1 alpha (6-keto-PGF1 alpha), the stable hydration product of prostacyclin (PGI2), were measured with radioimmunoassay during the first month of life in 25 preterm infants with idiopathic respiratory distress syndrome (IRDS) and 38 preterm controls wi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198210000-00005
更新日期:1982-10-01 00:00:00
abstract::The sp act of galactose-1-phosphate uridyltransferase has been measured in individual regions of adult rat brain to see if site-specific differences in enzyme activity can aid in the understanding of brain abnormalities observed in well-treated galactosemic patients. The sp act in the cerebellum, brain stem, and midbr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199205000-00021
更新日期:1992-05-01 00:00:00
abstract::We have previously shown that milk sensitization aggravates intestinal dysfunction in the malnourished guinea pigs, suggesting that it may also impair the recovery from malnutrition. To test this hypothesis, the growing guinea pigs were malnourished by feeding only maize for 7 d and then were refed for 21 d with a bal...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199812000-00018
更新日期:1998-12-01 00:00:00
abstract::In the original article, the legend within Fig. 3 incorrectly read as '*p < 0.10, **p < 0.05, ***p > 0.01'. This has now been changed to '*p < 0.10, **p < 0.05, ***p < 0.01'. This has been corrected in both the PDF and HTML versions of the Article. The authors would like to apologise for this error. ...
journal_title:Pediatric research
pub_type: 已发布勘误
doi:10.1038/s41390-019-0323-x
更新日期:2019-04-01 00:00:00
abstract::The effects of passive respiratory syncytial virus (RSV) immune serum given as preinoculation prophylaxis, postinoculation prophylaxis, and as therapy on d 5 after inoculation were evaluated in an illness model of RSV infection in BALB/c mice. Pre- and postinoculation prophylaxis reduced RSV replication in lung after ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199308000-00013
更新日期:1993-08-01 00:00:00
abstract:INTRODUCTION:Very early preterm infants (VPIs) are exposed to unpredictable noise in neonatal intensive care units. Their ability to perceive moderate acoustic environmental changes has not been fully investigated. RESULTS:Physiological values of the 598 isolated sound peaks (SPs) that were 5-10 and 10-15 dB slow-resp...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2011.76
更新日期:2012-04-01 00:00:00
abstract::We recently demonstrated that recombinant hGH exacerbates renal functional and structural injury in chronic puromycin aminonucleoside (PAN) nephropathy, an experimental model of glomerular disease. Therefore, we examined whether recombinant human (rh) IGF-I is a safer alternative for the treatment of growth failure in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199601000-00002
更新日期:1996-01-01 00:00:00
abstract::Children with cystic fibrosis excreted elevated urinary levels of all three polyamines--putrescine, spermidine, and spermine. Heterozygote parents excreted intermediate concentrations of the polyamines, but not levels significantly different from levels in normal controls. Patients with cystic fibrosis who were admini...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197910000-00011
更新日期:1979-10-01 00:00:00
abstract::Late Onset Central Hypoventilation Syndrome associated with Hypothalamic Dysfunction (LO-CHS/HD) is a distinct entity among the clinical and genetic heterogeneous group of patients with late onset central hypoventilation. Here we report a series of 13 patients with LO-CHS/HD. Rapid onset obesity is the first symptom o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318187dd0e
更新日期:2008-12-01 00:00:00
abstract::Deficient quantity of amniotic fluid causes fetal guinea pig lung hypoplasia. Oligohydramnios that lasts only 5 days in early gestation is sufficient to reduce fetal lung growth significantly. We quantitated lung structural alterations at 50 days gestation (term is 67 days) of fetal guinea pigs whose amniotic fluid wa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198610000-00013
更新日期:1986-10-01 00:00:00
abstract::The short-term (0-48 h) effects of maternal betamethasone administration on computerized fetal heart rate (FHR) parameters were studied in 36 pregnancies at increased risk for preterm delivery. FHR was recorded for 90 min immediately before the start of betamethasone treatment and again at 6-h intervals during the nex...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000155948.83570.EB
更新日期:2005-04-01 00:00:00
abstract::We examined the long-term effects of dietary protein restriction during rat pregnancy on serum IGF-I, serum IGF binding proteins, and liver IGF-I gene expression during postnatal development. Pregnant Wistar rats were fed ad libitum throughout gestation a normal (20% casein diet; P20 controls) or a low (5% casein; P5)...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199604000-00015
更新日期:1996-04-01 00:00:00
abstract:BACKGROUND:Early identification of infants at risk for complications from patent ductus arteriosus (PDA) may improve treatment outcomes. The aim of this study was to identify biochemical markers associated with persistence of PDA, and with failure of pharmacological treatment for PDA, in extremely preterm infants. MET...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-018-0182-x
更新日期:2019-09-01 00:00:00
abstract::Pearson's syndrome, a rare and fatal disorder characterized by refractory sideroblastic anemia and pancreatic insufficiency in infancy, is classified into mitochondrial cytopathies. To understand the molecular and genetic bases of this disorder, we have investigated the mitochondrial respiratory chain enzymes and the ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199307000-00024
更新日期:1993-07-01 00:00:00
abstract:BACKGROUND:To minimize secondary lung injury, ventilated preterm infants are extubated as soon as possible. To maximize extubation success, they are often placed in prone position. The effect of extubation and subsequent prone positioning on lung volumes is currently unknown. METHODS:Changes in end-expiratory lung vol...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1038/pr.2014.201
更新日期:2015-03-01 00:00:00
abstract::We have previously demonstrated that enterally administered heparin-binding EGF-like growth factor (HB-EGF) produced in Escherichia coli decreases the incidence and severity of intestinal injury in a neonatal rat model of necrotizing enterocolitis (NEC). In preparation for upcoming human clinical trials, large-scale p...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181994fa0
更新日期:2009-04-01 00:00:00
abstract::To examine uridine diphosphate hexose (UDPhexose) content of cells that have more complete metabolic patterns than erythrocytes, which have been commonly used in the study of galactosemia, the concentrations of uridine diphosphate galactose (UDPgalactose) and uridine diphosphate glucose (UDPglucose) were determined in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199411000-00014
更新日期:1994-11-01 00:00:00
abstract:BACKGROUND:Intrauterine growth restriction (IUGR) has been linked to heart disease in adulthood. Hence the IUGR heart is likely to be vulnerable to diabetic heart disease. The aim of this study was to examine the effect of induction of type 1 diabetes on myocardial collagen deposition and cardiac function in adult rats...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2012.94
更新日期:2012-10-01 00:00:00