The expression of epithelial-mesenchymal transition-related proteins in biliary epithelial cells is associated with liver fibrosis in biliary atresia.

Abstract:

BACKGROUND:The epithelial-mesenchymal transition (EMT) has been implicated as a key mechanism in the pathogenesis of liver fibrosis. The miR-200 family has been shown to inhibit EMT. METHODS:Liver fibrosis levels were assessed with Masson's trichrome staining of liver samples obtained from biliary atresia (BA) patients. The expressions of cytokeratin-7 (CK-7) and α-smooth muscle actin (α-SMA) in the liver sections were detected by immunohistochemical and immunofluorescent staining. EMTs were induced by transforming growth factor (TGF)-β1 in human biliary epithelial cells (BECs) in vitro. RESULTS:We showed that the EMT-related proteins CK-7 and α-SMA colocalized to the intrahepatic BECs in the liver sections of patients with BA. The level of α-SMA expression was related to liver fibrosis stage in BA. EMT in primary human intrahepatic BECs was induced by TGF-β1 in vitro. miR-200b is one member of the miR-200 family and significantly inhibited TGF-β1-mediated EMT in BECs. CONCLUSION:Together, these data suggest that the occurrence of EMT in BECs might contribute to BA fibrosis. miR-200b significantly affects the development and progression of TGF-β1-dependent EMT and fibrosis in vitro.

journal_name

Pediatr Res

journal_title

Pediatric research

authors

Xiao Y,Zhou Y,Chen Y,Zhou K,Wen J,Wang Y,Wang J,Cai W

doi

10.1038/pr.2014.181

subject

Has Abstract

pub_date

2015-02-01 00:00:00

pages

310-5

issue

2

eissn

0031-3998

issn

1530-0447

pii

pr2014181

journal_volume

77

pub_type

杂志文章
  • Neurosteroid replacement therapy using the allopregnanolone-analogue ganaxolone following preterm birth in male guinea pigs.

    abstract:BACKGROUND:Children born preterm, especially boys, are at increased risk of developing attention deficit hyperactivity disorder (ADHD) and learning difficulties. We propose that neurosteroid-replacement therapy with ganaxolone (GNX) following preterm birth may mitigate preterm-associated neurodevelopmental impairment. ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/s41390-018-0185-7

    authors: Shaw JC,Dyson RM,Palliser HK,Gray C,Berry MJ,Hirst JJ

    更新日期:2019-01-01 00:00:00

  • Utility of volatile organic compounds as a diagnostic tool in preterm infants.

    abstract:BACKGROUND:Volatile organic compounds (VOCs) are hydrocarbons that originate within different healthy and diseased tissues. VOCs can be secreted into the circulation and then excreted in the urine and faeces. In the lungs, VOCs are locally produced and can be detected in exhaled breath. VOCs can be identified using non...

    journal_title:Pediatric research

    pub_type: 杂志文章,评审

    doi:10.1038/s41390-020-0828-3

    authors: Wright H,Bannaga AS,Iriarte R,Mahmoud M,Arasaradnam RP

    更新日期:2020-03-02 00:00:00

  • Cerebral energy metabolism in phenylketonuria: findings by quantitative In vivo 31P MR spectroscopy.

    abstract::Both severe impairments of brain development in untreated infants and acute reversible neurotoxic effects on brain function are clinical features of phenylketonuria (PKU). For determining whether impairments of cerebral energy metabolism play a role in the pathophysiology of PKU, quantitative in vivo 31P magnetic reso...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/01.PDR.0000055867.83310.9E

    authors: Pietz J,Rupp A,Ebinger F,Rating D,Mayatepek E,Boesch C,Kreis R

    更新日期:2003-04-01 00:00:00

  • Salivary cortisol levels as a biomarker for severity of withdrawal in opioid-exposed newborns.

    abstract:BACKGROUND:Scoring tools used to quantify withdrawal in infants with neonatal abstinence syndrome (NAS) are often confounded by subjective measurements. This study assessed salivary cortisol as an objective biomarker of withdrawal severity in opioid-exposed newborns. METHODS:A prospective study was conducted in 25 ful...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/s41390-019-0601-7

    authors: Rodriguez N,Vining M,Bloch-Salisbury E

    更新日期:2020-05-01 00:00:00

  • Choline incorporation into lecithin in response to insulin or dexamethasone in homogeneous cell cultures of rat lung epithelial cells and fibroblasts.

    abstract::Labeled choline incorporation into adult rat lung alveolar epithelial cells and adult rat lung fibroblasts in monolayer culture was determined after incubation with insulin (Ins) 10 micrograms/ml, Dexamethasone (Dex) 10(-6)M, or no drug (ND). Incubation periods were 1, 3, 4, and 5 hours. The lecithin (phosphatidyl cho...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198007000-00014

    authors: Sharp MJ,Borer RC Jr,Vadnay L,Douglas WH

    更新日期:1980-07-01 00:00:00

  • Effect of ventilation on mechanical properties and pressure-flow relationships of immature airways.

    abstract::Ventilation of immature airways has been shown to result in pressure-induced deformation and alteration of mechanical properties. These changes in mechanical properties may alter the effect of compressive pressures on pressure-flow relationships. To test this hypothesis, unventilated (Group I, n = 8) and ventilated (g...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198805000-00017

    authors: Penn RB,Wolfson MR,Shaffer TH

    更新日期:1988-05-01 00:00:00

  • Influence of antenatal glucocorticoid on preterm lamb diaphragm.

    abstract::BackgroundPregnant women at a high risk of preterm delivery receive glucocorticoids to accelerate fetal lung maturation and surfactant synthesis. However, the effect of antenatal steroids on the developing diaphragm remains unclear. We hypothesized that maternal betamethasone impairs the fetal diaphragm, and the magni...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/pr.2017.99

    authors: Mahzabin T,Pillow JJ,Pinniger GJ,Bakker AJ,Noble PB,White RB,Karisnan K,Song Y

    更新日期:2017-09-01 00:00:00

  • Recent advances in primary immunodeficiencies: identification of novel genetic defects and unanticipated phenotypes.

    abstract::Primary immunodeficiencies (PIDs) have traditionally been defined according to their immunologic phenotype. Far from being concluded, the search for human genes that, when mutated, cause PID is actively being pursued. During the last year, four novel genetic defects that cause severe combined immunodeficiency and seve...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/PDR.0b013e31819dbe1e

    authors: Pessach I,Walter J,Notarangelo LD

    更新日期:2009-05-01 00:00:00

  • Intravenous lipid emulsions in the treatment of essential fatty acid deficiency: studies in young pigs.

    abstract::Essential fatty acid deficiency (EFAD) occurs in infants fed fat-free mixtures of glucose and amino acids. Although infusion of lipid emulsion rapidly reverses clinical symptoms, little is known about effects on tissue fatty acids. To study this question, five groups (n = 4/group) of neonatal pigs were studied. Three ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198412000-00027

    authors: Andersen DW,Filer LJ Jr,Stegink LD

    更新日期:1984-12-01 00:00:00

  • Effect of inspiratory resistive loading on costal and crural diaphragm electromyograms in piglets.

    abstract::We examined the effect of inspiratory resistive loaded breathing (IRL) on the electromyographic (EMG) activity of the costal and crural diaphragm in nine anesthetized spontaneously breathing piglets (age 10-23 days, weight 2.8-4.4 kg). Bipolar wire electrodes were inserted into the anterior paratendinous costal diaphr...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198701000-00007

    authors: Watchko JF,Mayock DE,Standaert TA,Woodrum DE

    更新日期:1987-01-01 00:00:00

  • Association of pulmonary surfactant protein A (SP-A) gene and respiratory distress syndrome: interaction with SP-B.

    abstract::Deficiency of the lipoprotein complex, surfactant, can lead to respiratory distress syndrome (RDS) in the prematurely born infant. The surfactant proteins (SP) play important roles in the function of surfactant. Previously, we have characterized four allelic variants of the SP-A1 gene (6A, 6A2, 6A3, and 6A4) and five ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-199802000-00003

    authors: Kala P,Ten Have T,Nielsen H,Dunn M,Floros J

    更新日期:1998-02-01 00:00:00

  • Magnesium deficiency anemia in the rat fetus.

    abstract::Magnesium-deficient fetuses exhibited malformations (44%), anemia, and edema. Maternal plasma magnesium levels at day 21 of pregnancy reflected the level of dietary magnesium (2.43 +/- 0.09 mg Mg/100 ml, control; 0.74 +/- 0.02 mg Mg/100 ml, deficient). Plasma magnesium levels of deficient fetuses showed similar decrea...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-197706000-00013

    authors: Cosens G,Diamond I,Theriault LL,Hurley LS

    更新日期:1977-06-01 00:00:00

  • Inhibitory effect of 5β-pregnane-3α,20β-diol on transcriptional activity and enzyme activity of human bilirubin UDP-glucuronosyltransferase.

    abstract::Prolonged unconjugated hyperbilirubinemia in infants associated with breast milk feeding is a common pediatric problem known as breast milk jaundice (BMJ). A polymorphic mutation (G71R) of bilirubin UDP-glucuronosyltransferase (UGT1A1) is a known cause of BMJ on the infantile side, but the responsible components of br...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/PDR.0b013e31822f242e

    authors: Ota Y,Maruo Y,Matsui K,Mimura Y,Sato H,Takeuchi Y

    更新日期:2011-11-01 00:00:00

  • Ultrastructure and function of alveolar macrophages from cystic fibrosis patients.

    abstract::Alveolar macrophages were isolated from three cystic fibrosis patients, and the structure and function of these cells were compared to that of normal alveolar macrophages. The cystic fibrosis (CF) and normal alveolar macrophages were able to phagocytize Pseudomonas in the presence of normal serum, but cells from both ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198005000-00003

    authors: Thomassen MJ,Demko CA,Wood RE,Tandler B,Dearborn DG,Boxerbaum B,Kuchenbrod PJ

    更新日期:1980-05-01 00:00:00

  • The effects of altered nutritional status upon insulin-like growth factors and their binding proteins in neonatal rats.

    abstract::To investigate the role of nutrition in the regulation of IGFs during the perinatal period, 10-d-old rats were infused intravenously with various concentrations of nutrients for 24 h. Breast-fed litter mates served as controls. The effect of caloric intake on concentrations of IGF-I and IGF-II as well as IGF-binding p...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198908000-00012

    authors: Philipps A,Drakenberg K,Persson B,Sjögren B,Eklöf AC,Hall K,Sara V

    更新日期:1989-08-01 00:00:00

  • Studies on requirements for amino acids in infants with disorders of amino acid metabolism. I. Effect of alanine.

    abstract::Two infants with disorders of propionate metabolism were studied at 7 months of age to determine optimum levels of intake of protein and calories to meet the requirements for essential amino acid for growth in infancy, and at the same time minimize the accumulation of toxic intermediates. An effect of alanine was foun...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198501000-00023

    authors: Kelts DG,Ney D,Bay C,Saudubray JM,Nyhan WL

    更新日期:1985-01-01 00:00:00

  • Synergistic effect of influenza a virus on endotoxin-induced mortality in rat pups: a potential model for sudden infant death syndrome.

    abstract::Sudden infant death syndrome is the most common cause of postneonatal infant mortality in the developed world. It is a diagnosis of exclusion with peak age of incidence between 2 and 6 mo. Fifty to 63% of these infants have a preexisting upper respiratory tract infection before death. We hypothesized that the immature...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-200210000-00005

    authors: Blood-Siegfried J,Nyska A,Lieder H,Joe M,Vega L,Patterson R,Germolec D

    更新日期:2002-10-01 00:00:00

  • Glucosylsphingosine accumulation in mice and patients with type 2 Gaucher disease begins early in gestation.

    abstract::Gaucher disease, the most common of the sphingolipidoses, results from the inherited deficiency of the enzyme glucocerebrosidase (EC 3.2.1.45). Although type 2 (acute neuronopathic) Gaucher disease is associated with rapidly progressive and fatal neurologic deterioration, the pathophysiologic mechanisms leading to the...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-200008000-00018

    authors: Orvisky E,Sidransky E,McKinney CE,Lamarca ME,Samimi R,Krasnewich D,Martin BM,Ginns EI

    更新日期:2000-08-01 00:00:00

  • Glucocorticoid receptor subunit expression in adenotonsillar tissue of children with obstructive sleep apnea.

    abstract::Tonsillectomy and adenoidectomy (T&A) is a frequent surgical procedure in children with obstructive sleep apnea (OSA). Many symptomatic children who do not fulfill the currently recommended criteria for T&A may benefit from topical intranasal steroid therapy. However, the expression of glucocorticoid receptor (GCR) ex...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/01.PDR.0000150722.34561.E6

    authors: Goldbart AD,Veling MC,Goldman JL,Li RC,Brittian KR,Gozal D

    更新日期:2005-02-01 00:00:00

  • Oxygen desaturations in the early neonatal period predict development of bronchopulmonary dysplasia.

    abstract:BACKGROUND:Bradycardia and oxygen desaturation episodes are common among preterm very low birth weight (VLBW) infants in the Neonatal Intensive Care Unit (NICU), and their association with adverse outcomes such as bronchopulmonary dysplasia (BPD) is unclear. METHODS:For 502 VLBW infants we quantified bradycardias (HR ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/s41390-018-0223-5

    authors: Fairchild KD,Nagraj VP,Sullivan BA,Moorman JR,Lake DE

    更新日期:2019-06-01 00:00:00

  • Surface cooling rapidly induces coordinated activity in the upper and lower airway muscles of the fetal lamb in utero.

    abstract::Stimulation of cutaneous thermoreceptors may be an important factor in the initiation of continuous breathing at birth. Maintenance of a patent airway is also important in the continuation of effective ventilation after birth, but whereas in the adult the principal pharyngeal dilator is the genioglossus muscle, in the...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/00006450-198803000-00005

    authors: Johnston BM,Gunn TR,Gluckman PD

    更新日期:1988-03-01 00:00:00

  • Investigating the effects of cerebrospinal fluid removal on cerebral blood flow and oxidative metabolism in infants with post-hemorrhagic ventricular dilatation.

    abstract::BackgroundPost-hemorrhagic ventricular dilatation (PHVD) is predictive of mortality and morbidity among very-low-birth-weight preterm infants. Impaired cerebral blood flow (CBF) due to elevated intracranial pressure (ICP) is believed to be a contributing factor.MethodsA hyperspectral near-infrared spectroscopy (NIRS) ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/pr.2017.131

    authors: McLachlan PJ,Kishimoto J,Diop M,Milej D,Lee DSC,de Ribaupierre S,St Lawrence K

    更新日期:2017-10-01 00:00:00

  • Airway smooth muscle changes in the nitrofen-induced congenital diaphragmatic hernia rat model.

    abstract::In the fetal rat, nitrofen induces congenital diaphragmatic hernia (CDH) and pulmonary vascular remodeling similar to what is observed in the human condition. Airway hyperactivity is common in infants with CDH and attributed to the ventilator-induced airway damage. The purpose of this study was to test the hypothesis ...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/01.PDR.0000057986.74037.7B

    authors: Belik J,Davidge ST,Zhang W,Pan J,Greer JJ

    更新日期:2003-05-01 00:00:00

  • Physical activity and sedentary levels in children with juvenile idiopathic arthritis and inflammatory bowel disease. A systematic review and meta-analysis.

    abstract:BACKGROUND:Physical activity (PA) is essential for children throughout their growth and maturation. It improves physiological and psychological health and limits the risk of developing metabolic disorders. However, some chronic physiological and metabolic diseases may lead to decreased PA. The diversity of outcomes in ...

    journal_title:Pediatric research

    pub_type: 杂志文章,meta分析

    doi:10.1038/s41390-019-0409-5

    authors: Bourdier P,Saidi O,Rochette E,Ratel S,Merlin E,Pereira B,Duché P

    更新日期:2019-08-01 00:00:00

  • Dual-specificity phosphatase (DUSP) genetic variants predict pulmonary hypertension in patients with bronchopulmonary dysplasia.

    abstract:BACKGROUND:Pulmonary hypertension (PH) in patients with bronchopulmonary dysplasia (BPD) results from vasoconstriction and/or vascular remodeling, which can be regulated by mitogen-activated protein kinases (MAPKs). MAPKs are deactivated by dual-specificity phosphatases (DUSPs). We hypothesized that single-nucleotide p...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1038/s41390-019-0502-9

    authors: Chen LL,Zmuda EJ,Talavera MM,Frick J,Brock GN,Liu Y,Klebanoff MA,Trittmann JK

    更新日期:2020-01-01 00:00:00

  • Glucose kinetics in glucose-infused small for gestational age infants.

    abstract::To evaluate the maturation of glucose homeostasis in the small for gestational age (SGA) neonate, glucose kinetics were measured with 78% enriched D-[U-13C]glucose by the prime plus constant infusion technique in nine SGA infants and compared with the rate obtained in seven term appropriate for gestational age infants...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:

    authors: Cowett RM,Susa JB,Oh W,Schwartz R

    更新日期:1984-01-01 00:00:00

  • Influence of intravenous sildenafil on cerebral oxygenation measured by near-infrared spectroscopy in infants after cardiac surgery.

    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

    authors: Nagdyman N,Fleck T,Bitterling B,Ewert P,Abdul-Khaliq H,Stiller B,Hübler M,Lange PE,Berger F,Schulze-Neick I

    更新日期:2006-03-01 00:00:00

  • Gestational age, sex and maternal parity correlate with bone turnover in premature infants.

    abstract::Factors affecting bone turnover in premature infants are not entirely clear but certainly are different from those influencing bones of adults and children. To identify fetal and maternal factors that might influence bone turnover, we prospectively studied 50 infants (30 preterm and 20 full-term) born at Ain Shams Uni...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/01.PDR.0000160591.70409.C8

    authors: Aly H,Moustafa MF,Amer HA,Hassanein S,Keeves C,Patel K

    更新日期:2005-05-01 00:00:00

  • The promise of a prophylactic Epstein-Barr virus vaccine.

    abstract::The worldwide burden of disease due to Epstein-Barr virus (EBV) infection is enormous. Diseases include endemic Burkitt lymphoma, infectious mononucleosis, cancers after transplantation, Hodgkin lymphoma, and nasopharyngeal carcinoma. A prophylactic EBV vaccine has the potential to significantly reduce the incidence a...

    journal_title:Pediatric research

    pub_type: 杂志文章,评审

    doi:10.1038/s41390-019-0591-5

    authors: Balfour HH Jr,Schmeling DO,Grimm-Geris JM

    更新日期:2020-01-01 00:00:00

  • Effect of insulin with concurrent amino acid infusion on protein metabolism in rapidly growing pubertal children with type 1 diabetes.

    abstract::Insulin treatment of prepubertal children with insulin-dependent diabetes improves body protein balance by decreasing the rate of protein degradation without stimulating protein synthesis. However, insulin also causes hypoaminoacidemia, so the inability of insulin to stimulate protein synthesis may have been limited b...

    journal_title:Pediatric research

    pub_type: 杂志文章

    doi:10.1203/01.PDR.0000169976.20029.64

    authors: Godil MA,Wilson TA,Garlick PJ,McNurlan MA

    更新日期:2005-08-01 00:00:00