Abstract:
:The previous biochemical evidence had suggested that glutaric aciduria type II (GA II) is due to deficient dehydrogenation of multiple short-chain acyl coenzyme A's (CoA's), bu the precise biochemical mechanism underlying this disease was unknown. We investigated substrate oxidation and in vitro activities of isovaleryl CoA- and butyryl CoA dehydrogenases as well as that of electron-transferring flavoprotein (ETF) in cultured skin fibroblasts from a patient with GA II. GA II cells have a markedly decreased ability to oxidize [1-14C]butyrate, [2-14C]lysine, and [2,14C]leucine (3, 9, and 9% of control values, respectively). Mitochondrial isovaleryl CoA- and butyryl CoA dehydrogenase activities in GA II cells were determined using a tritium release assay with [2,3-3H] acyl-CoA's as substrate. When an artificial electron acceptor, phenazine methosulfate (PMS) was not added in the assay media, these activities were 108 and 113% of controls, respectively. This represents the normal abilities of the dehydrogenases in GA II cells to bind the substrate and to catalyze tritium exchange between the bound substrate and solvent. When PMS was added to the assay mixture, these activities were 88 and 70% of control values, respectively, indicating that these enzymes can both dehydrogenate their substrates normally and then transfer electrons to an acceptor (PMS). ETF activity in mitochondrial sonic supernatants from GA II cells, as assessed by a newly devised method, was 159% of control values. These observations suggest that the acyl CoA dehydrogenases themselves and ETF are not defective in GA II. Therefore, the deficiency of another common gene product necessary for the function of all the affected acyl CoA dehydrogenases must be sought to explain the etiology of GA II.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Rhead W,Mantagos S,Tanaka Kdoi
10.1203/00006450-198012000-00013subject
Has Abstractpub_date
1980-12-01 00:00:00pages
1339-42issue
12eissn
0031-3998issn
1530-0447journal_volume
14pub_type
杂志文章abstract::The response of cerebral blood volume (CBVR) to a small induced change in arterial carbon dioxide tension was studied by near-infrared spectroscopy in 17 newborn infants born from 26 wk of gestation to term. All 17 infants were undergoing mechanical ventilation but had apparently normal brains. The CBVR per kPa change...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199106010-00007
更新日期:1991-06-01 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::Genetic factors are implicated in pathogenesis of neonatal hyperbilirubinemia. In this nested case-control study, we determined 1) frequency of thymine-adenine (TA)n promoter polymorphism and Gly71Arg mutation in uridine diphosphoglucuronate-glucuronosyltransferase 1A1 (UGT1A1) gene in neonates > or =35-wk gestation p...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31819ed5de
更新日期:2009-06-01 00:00:00
abstract::Previously, we have reported marked pulmonary inflammation in infants who develop chronic lung disease of prematurity. We revisited these infants who did not have clinical or laboratory evidence of infection and searched for Ureaplasma urealyticum, group B streptococci, and other microbes by reverse transcription-PCR ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000100757.38675.50
更新日期:2004-01-01 00:00:00
abstract::Many attempts have been made to obtain safe and effective analgesia in newborns. Oral glucose-water has been found to have analgesic properties in neonates. We investigated whether other sensory stimulation added to oral glucose provided more effective analgesia than oral glucose alone. In a randomized prospective dou...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1203/00006450-200204000-00010
更新日期:2002-04-01 00:00:00
abstract::Cerebral blood flow (CBF) was measured by means of Celabeled microspheres in infant (20-day-old) and adult (3-month-old) rats, anesthetised with Na-5-ethyl-5-(1-methylpropyl)2-thiobarbituric acid. Cerebral arteriovenous differences of acetoacetate, D-beta-hydroxybutyrate, glucose, lactate, and oxygen and brain DNA con...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197611000-00002
更新日期:1976-11-01 00:00:00
abstract:BACKGROUND:A growing body of evidence defines inflammation as a hallmark feature of disease pathogenesis of Duchenne muscular dystrophy. To tailor potential immune modulatory interventions, a better understanding of immune dysregulation in Duchenne muscular dystrophy is needed. We now asked whether dystrophin deficienc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0427-3
更新日期:2019-08-01 00:00:00
abstract::The relationship between cerebral blood flow (CBF) and the evolution of brain edema was investigated in an experimental model of perinatal hypoxic-ischemic brain damage. Seven-d postnatal rats were subjected to unilateral common carotid artery ligation followed by 3 h of hypoxia with 8% oxygen at 37 degrees C. This in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199005000-00007
更新日期:1990-05-01 00:00:00
abstract::In preterm infants, low levels of insulin like growth factor 1 (IGF-I) have been associated with impaired growth and retinopathy of prematurity. Our objective was to study safety and pharmacokinetics of i.v. administered rhIGF-I with its binding protein 3 (rhIGFBP-3) to preterm infants. At 3 d chronological age, an i....
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/PDR.0b013e31819d9e8c
更新日期:2009-05-01 00:00:00
abstract:BACKGROUND:Intrauterine growth restriction (IUGR) increases the risk of adult-onset hypercholesterolemia. High-fat diet (HFD) consumption potentiates IUGR-induced increased cholesterol. Cholesterol is converted to bile acids by Cyp7a1 in preparation for excretion. We hypothesized that IUGR rats fed a HFD will have incr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.117
更新日期:2014-11-01 00:00:00
abstract:BACKGROUND:Clinicians could modify dietary interventions during early infancy by monitoring fat and fat-free mass accretion in very preterm infants. METHODS:Preterm infants were randomly assigned to either having reports on infant body composition available to the clinicians caring for them (intervention group) or not...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-1052-x
更新日期:2020-11-01 00:00:00
abstract::This investigation was undertaken to determine the nature of acute alterations in renal function following the production of hypoxemia, hypercarbia, and acidosis in newborn piglets 6-96 hr of age. After completion of the surgical procedure piglets were allowed to recover from the effects of anesthesia. When respirator...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197803000-00013
更新日期:1978-03-01 00:00:00
abstract::Accurate interpretation of the results of GH stimulation tests is of pivotal importance not only in the evaluation of the etiology of growth retardation in children but also in the selection of the best candidates for GH therapy. We performed this study to test a novel immunofunctional GH ( IFGH) assay that makes use ...
journal_title:Pediatric research
pub_type: 评论,杂志文章
doi:10.1203/00006450-200011000-00010
更新日期:2000-11-01 00:00:00
abstract::Studies in adult animal and human subjects have suggested that the methylxanthine drugs can delay the onset or attenuate the severity of diaphragmatic fatigue. We have investigated the effect of aminophylline and caffeine on the pressure-generating capacity of the fatigued diaphragm in 1-mo-old piglets. Measurements o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199211000-00019
更新日期:1992-11-01 00:00:00
abstract::Adverse perinatal events affecting cerebral functions are a major cause of neonatal mortality, morbidity, and long-term neurologic deficit. Intrapartum fetal EEG, which records fetal brain electrical activity, provides a monitoring modality for evaluating the fetal CNS during labor. In this study, we describe a new ap...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200009000-00013
更新日期:2000-09-01 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::Acute hemolytic anemia associated with red blood cell (RBC) glucose-6-phosphate dehydrogenase (G6PD) deficiency is commonly encountered in the Mediterranean basin. Nevertheless, concomitant clinical evidence of white blood cell G6PD deficiency is extremely rare in Israel. This study sought to assess simultaneously lev...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000120680.47846.47
更新日期:2004-05-01 00:00:00
abstract:BACKGROUND:Near-infrared spectroscopy (NIRS) is an upcoming clinical method for monitoring regional cerebral oxygen saturation (rScO2) in neonates. There is a growing market offering different devices and sensors. Even though this technique is increasingly clinically applied, little is known about the similarities and/...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.133
更新日期:2013-11-01 00:00:00
abstract:BACKGROUND:Serum caffeine concentrations >20 μg/ml (100 μmol/l) in infants treated for apnea of prematurity increases TNF-α and decreases IL-10, changes that perhaps are linked to comorbidities. We hypothesize that this proinflammatory cytokine profile may be linked to differential binding of caffeine to adenosine rece...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.50
更新日期:2016-07-01 00:00:00
abstract::Leptin, a hormone secreted by adipocytes, is elevated in blood of obese adults. It is unknown whether the concentration is affected by gender, ethnicity, age, or stage of sexual maturation in children. We measured serum leptin levels in 183 children and 27 young adults using a double-antibody ELISA assay. Body fat mas...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199710000-00010
更新日期:1997-10-01 00:00:00
abstract::Sleep organization of infants may be influenced by differences in nutrient intakes from human milk and formula. Because sleep/awake and sleep stage patterns affect energy expenditure, we hypothesized that differences in sleep organization between breast-fed and formula-fed infants might account in part for differences...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199211000-00003
更新日期:1992-11-01 00:00:00
abstract::Thrombocytopenia in the first days of life, in association with evidence of reduced megakaryocytopoiesis and platelet production at birth, is common in sick preterm babies. Thrombopoietin (Tpo) is the major regulator of platelet production in adults. However, these babies have low Tpo levels at birth, suggesting that ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199907000-00005
更新日期:1999-07-01 00:00:00
abstract::Evidence is inconsistent or poorly understood for links between polychlorinated biphenyls (PCBs), 1,1'-dichloro-2,2'-bis(4-chlorophenyl)ethylene (DDE), and organophosphate (OP) pesticides and adverse pregnancy outcomes, although they are known developmental toxicants. We measured biomarkers of maternal exposure to DDE...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e31802d77f0
更新日期:2007-02-01 00:00:00
abstract:BACKGROUND:Bronchopulmonary dysplasia (BPD) is the most common and serious chronic lung disease of premature infants. Connective tissue growth factor (CTGF) plays an important role in tissue development and remodeling. We have previously shown that targeted overexpression of CTGF in alveolar type II epithelial cells re...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.52
更新日期:2016-07-01 00:00:00
abstract::Extracellular glutathione peroxidase (E-GPx) is a selenoenzyme that reduces hydrogen peroxide and organic peroxides. All plasma glutathione peroxidase (GPx) activity in humans is attributable to E-GPx. The gastrointestinal (GI) tract also synthesizes and secretes E-GPx into the extracellular milieu. Endogenously gener...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200205000-00016
更新日期:2002-05-01 00:00:00
abstract:BACKGROUND:Neonatal seizures can result in chronic epilepsy and long-term behavioral and cognitive deficits. Levetiracetam (LEV), an antiepileptic drug that binds to the synaptic vesicle protein 2A (SV2A), has been increasingly used off-label for the therapy of neonatal seizures. Preclinical data regarding the acute or...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2012.151
更新日期:2013-01-01 00:00:00
abstract::In muscle, sepsis reduces protein synthesis (MPS) by restraining translation in neonates and adults. Even though protein accretion decreases with development as neonatal MPS rapidly declines by maturation, the changes imposed by development on the sepsis-associated decrease in MPS have not been described. Pigs at 7 an...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3182176da1
更新日期:2011-06-01 00:00:00
abstract:BACKGROUND:Aminoglycoside exposure is a common cause of acute kidney injury (AKI). Delay in the diagnosis of AKI using conventional biomarkers has been one of the important obstacles in applying early effective interventions. We tested the hypothesis that urinary metabolomics could identify novel early biomarkers for t...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.34
更新日期:2013-05-01 00:00:00
abstract::The newborn immune system differs quantitatively and functionally from that of adults. Development of the immune system has important implications for childhood diseases. The immaturity of the immune system in the first years of life may contribute to failure of tolerance induction and in the development of allergic d...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/00006450-199609000-00001
更新日期:1996-09-01 00:00:00
abstract::Developmental disability, intrauterine growth retardation, renal anomalies, and dysmorphic features have been described in offspring of women who abuse toluene during pregnancy. A Sprague-Dawley rat model was developed to study this clinical syndrome. During d 6-19 of gestation, 11 treated dams received daily gavage d...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199412000-00023
更新日期:1994-12-01 00:00:00