Abstract:
BACKGROUND:Hirschsprung disease (HSCR) is the third most common congenital disorder of the gastrointestinal tract. This study aims to elucidate changes in protein expression between the normal and aganglionic hindgut in human HSCR. METHODS:The biopsies were obtained from the normal and aganglionic hindgut in human HSCR, and the comparative proteomics were analyzed by mass spectrometry (MS)-based two-dimensional gel electrophoresis (2DE). RESULTS:A total of 932-986 protein spots were identified in each of the gut segments, among which 30 spots had at least an eightfold difference in volume (%). Of the 30 differentially expressed spots, 15 proteins were identified via sequence analysis. Among these 15 proteins, eight were upregulated and seven were downregulated in the aganglionic group. The well-represented classes included biomarkers of enteric ganglions, extracellular matrix proteins, LIM domain proteins, serum proteins, and other pleiotropic proteins. Five proteins were selected and verified by western blotting and real-time PCR, and the results were consistent with the results of 2DE. CONCLUSION:MS-based 2DE can help to identify pathological relevant proteins in HSCR; it defines an extensive protein catalog of the normal and aganglionic hindgut and may constitute the basis to understand pathophysiological mechanisms related to the HSCR.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Zhang SC,Chen F,Jiang KL,Yuan ZW,Wang WLdoi
10.1038/pr.2014.33subject
Has Abstractpub_date
2014-06-01 00:00:00pages
754-61issue
6eissn
0031-3998issn
1530-0447pii
pr201433journal_volume
75pub_type
杂志文章abstract::Nitric oxide plays a major role in vascular tone control. Increased blood levels of bradykinin (BK), which stimulates nitric oxide biosynthesis, occur at birth. BK effects on ductus arteriosus (DA) tone were investigated in fetal rabbit under fetal (2.5% O2 "low PO2") and neonatal (30% O2 "high PO2") conditions using ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199904010-00017
更新日期:1999-04-01 00:00:00
abstract::The use of tissue engineering to develop new urinary bladder could have direct impact on children suffering from congenital bladder dysfunction. Current bladder tissue engineering protocols rely on scaffolds to support the ingrowth of new cells and the subsequent development of new tissues. The interaction between cel...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e3181d35034
更新日期:2010-05-01 00:00:00
abstract::Studies have shown that human neonates who develop group B streptococcal sepsis usually lack opsonic antibody (Ab) to their infecting strain and that these neonates may also have impaired polymorphonuclear leukocyte (PMN) function. The present study was designed to determine the efficacy of administration of PMNs or o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198012000-00031
更新日期:1980-12-01 00:00:00
abstract::The major variation in effect between undiluted cystic fibrosis and heterozygote sera on rabbit tracheal epithelium was in terms of the time after exposure at which changes occurred. Exposure to both types of sera for 3 min caused release of goblet cell mucus and ciliary resorption possibly by apical swelling of colum...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198011000-00005
更新日期:1980-11-01 00:00:00
abstract::We established an experimental animal model of the gastrointestinal colonization and systemic disease following oral challenge of type III group B streptococcal strain in 3-day-old newborn rats. Two type III group B streptococcal strains isolated from the cerebrospinal fluid of septic newborn infants produced coloniza...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198412000-00022
更新日期:1984-12-01 00:00:00
abstract::We investigated the early postnatal changes of the mesenteric circulation and its relation to the systemic circulation in 15 preterm infants. The infants were studied before the first feeding on d 1 and pre- and postprandially on d 3, 4, 5, and 7. Blood flow velocity was measured by ultrasound Doppler in the superior ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199602000-00015
更新日期:1996-02-01 00:00:00
abstract::Liposomes can be used for the delivery of drugs in cancer chemotherapy. After i.v. injection liposomes are to a large extent taken up by the mononuclear phagocytic system (MPS). When treating diseases in the MPS, such as the histiocytic syndromes, this property is of potential value for drug targeting and may lead to ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199507000-00002
更新日期:1995-07-01 00:00:00
abstract::The effect of diet, human milk or formula, on gastric function (lipase and pepsin activity, pH, and volume) and intragastric digestion of fat was assessed in 28 appropriate for gestational age preterm infants (gestational age, 28.9 +/- 1.4, 29.1 +/- 0.9, 29.5 +/- 0.6 wk; birth weight, 1.00 +/- 0.14 to 1.18 +/- 0.07 kg...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1203/00006450-199609000-00011
更新日期:1996-09-01 00:00:00
abstract::Prothrombin of newborns was investigated by means of the quantitative immunoelectrophoresis and the two-dimensional immunoelectrophoresis. The plasma of 10 of 24 healthy term newborns and of 5 preterm infants taken immediately after birth showed one prothrombin precipitate in the alpha-2-beta-globulin position when a ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197911000-00011
更新日期:1979-11-01 00:00:00
abstract::Seven essentially healthy term infants who received gentamicin starting on the 1st day of life for prolonged rupture of membranes and maternal fever were compared with nine healthy term infants to determine whether this drug induces alterations in the auditory pathway. The auditory pathway was studied on the 3rd day o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199009000-00017
更新日期:1990-09-01 00:00:00
abstract:BACKGROUND:The prevalence of vitamin D (vitD) deficiency presenting as rickets is increasing worldwide. Insufficient sun exposure, vitD administration, and/or calcium intake are the main causes. However, vitD system-related genes may also have a role. METHODS:Prospective study: 109 rachitic children completed a 6-mo s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.222
更新日期:2017-02-01 00:00:00
abstract::A rabbit model of group B Streptococcal (GBS) shock was used to determine if myocardial dysfunction contributes to GBS shock and, if so, to ascertain if prostaglandins modulate this dysfunction. The infusion of heat-killed GBS (group I) produced a dramatic decrease in the first derivative of left ventricular pressure ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198506000-00001
更新日期:1985-06-01 00:00:00
abstract::The ventricular epicardial activation sequence (VEAS) in 15 anesthetized lambs (near term fetus to 3.5 months of age) was determined using 40 simultaneously recorded bipolar electrograms for each animal. Isochrone maps were drawn by hand using relative activation times determined from the maximum first derivative of m...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
abstract::We have shown that vitamin A (VA) and retinoic acid (RA) synergistically increase lung retinyl ester content in neonatal rats. To confirm whether this biochemical synergism attenuates early neonatal hyperoxic lung injury in mice, we exposed newborn C57BL/6 mice to 95% O2 or air from birth to 4 d. The agent [vehicle, V...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181dbac3d
更新日期:2010-06-01 00:00:00
abstract::Groups of newborn rats were transthoracically inoculated with 1 X 10(6) type III group B streptococci/g body wt, either alone or in combination with 1.5 microgram/g body wt of type-specific antibody derived from hybridoma cell lines. Ninety-four percent of the animals who received bacteria alone died. In contrast, non...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198310000-00005
更新日期:1983-10-01 00:00:00
abstract:BACKGROUND:Hurricane Maria struck Puerto Rico on 20 September 2017 causing catastrophic devastation. Prolonged shortage of food had been a substantial challenge to the residents after Maria. Experiencing food insecurity in utero has been associated with negative health outcomes later in life. We aim to examine whether ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0839-0
更新日期:2020-12-01 00:00:00
abstract::Little is known about factors determining height outcome during GH treatment in Turner syndrome (TS). We investigated 987 TS children within the Kabi International Growth Study (KIGS) who had reached near adult height (NAH) after >4 y GH treatment (including >1 y before puberty). Through multiple regression analysis w...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000250039.42000.c9
更新日期:2007-01-01 00:00:00
abstract::To localize fetal rabbit lung beta-adrenoreceptors before and after glucocorticoid treatment, light microscopic autoradiography was performed with the reversible radiolabeled beta-adrenergic antagonist, [3H]dihydroalprenolol, on day 26 of gestation. Autoradiograms of adult lung and fetal myocardium were also prepared....
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198411000-00028
更新日期:1984-11-01 00:00:00
abstract::Basal and pentagastrin-stimulated gastric acid secretion was measured in 26 healthy newborn infants on the 1st and 2nd days of life. Basal acid outputs (BAO) on day 1 (0.378 mEq/hr) and day 2 (0.388 mEq/hr) were similar and not significantly different from the maximal acid outputs (MAO) on day 1 (0.413 mEq/hr) and day...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197901000-00008
更新日期:1979-01-01 00:00:00
abstract::A study using fractional clearances during orally induced water diuresis was designed to delineate the mechanism underlying defective tubular reabsorption of sodium in very low-birth-weight neonates. The use of clearance methodology during maximal water diuresis may give an indirect estimate of distal sodium delivery ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198312000-00017
更新日期:1983-12-01 00:00:00
abstract::Human GH is encoded by the GH-1 gene which belongs to the GH gene cluster encompassing a distance of about 65 kb on the long arm of chromosome 17. Familial isolated growth hormone deficiency (IGHD) is associated with at least four Mendelian disorders. These include two forms that have autosomal recessive inheritance (...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199801000-00016
更新日期:1998-01-01 00:00:00
abstract::Studies using stable isotopically labeled glucose and palmitate as precursors of pulmonary surfactant synthesis have demonstrated slow surfactant turnover in premature infants with respiratory distress syndrome (RDS). However, only limited data about surfactant turnover are available for term infants. Because acetate ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000075084.56255.E0
更新日期:2003-08-01 00:00:00
abstract::Clinical studies have suggested that the early stages of bilirubin encephalopathy are reversible. These phenomena are investigated at the cellular level using the neuroblastoma cell line N-115 as a model system. To determine whether the cell line N-115 can recover from bilirubin toxicity, and whether the cellular func...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198904000-00012
更新日期:1989-04-01 00:00:00
abstract::Intrahepatic and intracerebral metabolic responses to neonatal fasting or enteric carbohydrate alimentation were investigated among newborn dogs. Pups were either fasted or given an intravenous glucose infusion (alimented) before an enteric feeding of physiologic quantities of either glucose or galactose. These pups w...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198304000-00012
更新日期:1983-04-01 00:00:00
abstract::Vitamin K deficiency is a relatively common condition in neonates. However, the role of vitamin K in neonatal bone metabolism remains to be determined. Osteocalcin (OC) is the most abundant noncollagenous protein in bone, and is regulated to be gamma-carboxylated by vitamin K. In this study, we measured gamma-carboxyl...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200211000-00009
更新日期:2002-11-01 00:00:00
abstract::Pulmonary surfactant is inactivated in meconium aspiration syndrome and neonatal pneumonia. Development of an exogenous surfactant less sensitive to inactivation might be useful for treating these diseases. We investigated in vitro whether addition of the cationic cyclic membrane cross-linking peptide polymyxin B (PxB...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000200806.32822.e6
更新日期:2006-03-01 00:00:00
abstract::The copper concentrations in organs of developing Long-Evans Cinnamon (LEC) rats (2 d to 13 mo) were measured to elucidate the pathogenesis of their hereditary hepatitis. Hepatic copper contents of LEC rats were significantly higher than those of control rats (26 to 92 times higher). The subcellular distribution of he...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199203000-00011
更新日期:1992-03-01 00:00:00
abstract::Escherichia coli which elaborate heat stable enterotoxin (ST) are a major cause of endemic diarrhea in infants. The reason(s) for this increased susceptibility of infants to ST-mediated diarrhea is unknown. We investigated the possibility that the immature (14 and 21 day old) rat small intestine is more sensitive to S...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00017
更新日期:1986-06-01 00:00:00
abstract::In this study, we have investigated the possible role of the pro-aggregatory arachidonic acid (AA) metabolite thromboxane, in the impaired function of neonatal platelets. In platelet-rich plasma thromboxane production (measured by radioimmunoassay of thromboxane B2) was not different between neonates and adults when s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198409000-00003
更新日期:1984-09-01 00:00:00
abstract::The visual evoked potential (VEP) of five healthy, near-term exteriorized fetal lambs was compared with the VEP obtained from the same animals during the first hour after ventilation. Characteristics of the individual wave components and their relationship to blood pressure, heart rate, and arterial blood gas measurem...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198111000-00009
更新日期:1981-11-01 00:00:00