Abstract:
:Carnitine palmitoyltransferase (CPT) consists of two activities located in the outer (CPT I) and the inner (CPT II) mitochondrial membranes. CPT II deficiency in the adult as well as in the infantile form of the disease has been shown to result from mutations in the CPT II cDNA. Nothing is known regarding the genetic defect in CPT I deficiency. We carried out complementation experiments between CPT I- and infantile CPT II-deficient cell lines. Restoration of 3H2O release from [9,10(n)-3H]-palmitate was chosen as criterion of complementation. As expected, no complementation was observed in heteropolykaryons resulting from fusions between CPT II-deficient cells. Similar results were obtained in fusions between CPT I-deficient cells, suggesting that the enzymatic defect in these cell lines results from mutations in the same gene. Conversely, complementation was observed in fusions between CPT I- and CPT II-deficient cells. These data support that CPT I and CPT II defects result from mutations in distinct genes. Palmitate oxidation by control or CPT I-deficient cell lines was decreased when cocultured with infantile CPT II-deficient cell lines. This effect, not observed in coculture including an adult CPT II-deficient cell line, was carnitine-dependent. The possible mechanism of this effect, suppressed by a high carnitine concentration, is discussed.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Slama A,Brivet M,Boutron A,Legrand A,Saudubray JM,Demaugre Fdoi
10.1203/00006450-199610000-00005subject
Has Abstractpub_date
1996-10-01 00:00:00pages
542-6issue
4eissn
0031-3998issn
1530-0447journal_volume
40pub_type
杂志文章abstract::Oxidant stress has been implicated as playing a role in the pathogenesis of cholestatic liver injury. The objective of this study was to determine whether the xanthine oxidase/xanthine dehydrogenase enzyme system was involved in this oxidant stress. Adult Sprague-Dawley rats were treated with the xanthine oxidase inhi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199809000-00022
更新日期:1998-09-01 00:00:00
abstract:BACKGROUND:Neonates with hypoxic-ischemic encephalopathy (HIE) frequently develop acute kidney injury (AKI). Aminophylline has been shown to reduce severe renal dysfunction in neonates after perinatal asphyxia. However, the effect of aminophylline on renal function in neonates undergoing hypothermia has not been studie...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0999-y
更新日期:2020-06-05 00:00:00
abstract::Lung distensibility and airway mechanics were evaluated in 24 asthmatic children and adolescents, ages between 7 and 21 years, by quasi-static pressure-volume curves and by the static recoil-lung conductance relationship. The measurements were obtained by the step-wise inflation technique and the pressure-volume curve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198411000-00020
更新日期:1984-11-01 00:00:00
abstract::Neonatal PMN (polymorphonuclear neutrophils) exhibit altered inflammatory responsiveness and greater longevity compared with adult PMN; however, the involved mechanisms are incompletely defined. Receptors containing immunoreceptor tyrosine-based inhibitory motif (ITIM) domains promote apoptosis by activating inhibitor...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181b1bc19
更新日期:2009-09-01 00:00:00
abstract:INTRODUCTION:The Special Supplemental Nutrition Program for Women, Infants, and Children (WIC) serves 50% of infants and 25% of preschool-aged children in the United States and collects height and weight measurements from eligible children every 6 mo, making WIC data a valuable resource for studying childhood growth an...
journal_title:Pediatric research
pub_type: 杂志文章,多中心研究
doi:10.1038/pr.2011.37
更新日期:2012-03-01 00:00:00
abstract::We used quin2, a high affinity fluorescent calcium indicator to measure free cytosolic Ca++ in neutrophils isolated from fresh heparinized blood of 13 cystic fibrosis (CF) patients (age range 6-32 yr) and 11 healthy individuals (age range 25-41 yr) as controls. The mean value of free cytosolic Ca++ for CF patients was...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198519040-00006
更新日期:1985-04-01 00:00:00
abstract::Continuous EEG monitoring has not been used widely in neonatal intensive care, especially in the care of extremely premature infants, probably in part because of a lack of a reliable quantitative method. The purpose of this study was to quantify the EEG of the very premature infants just after birth by using spectral ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000153868.77623.43
更新日期:2005-03-01 00:00:00
abstract::To understand scoliosis, related comorbidities, and phenotype-genotype correlations in individuals with Rett syndrome (RTT), the Rare Disease Clinical Research Network database for RTT was probed. Clinical evaluations included a detailed history and physical examination, comprehensive anthropometric measurements, and ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181d0187f
更新日期:2010-04-01 00:00:00
abstract:INTRODUCTION:Corticosteroids have been widely used in clinical medicine as a first-line therapy to modify the inflammatory response in many pulmonary and systemic diseases. Inhaled and intratracheally administered corticosteroids have a particular interest in that their use allows the clinician to circumvent systemic s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2011.78
更新日期:2012-04-01 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::Jejunal biopsies obtained from 45 children referred for a variety of clinical conditions were examined histologically and by standard immunofluorescence methods. The number of plasma cells was recorded per "mucosal tissue unit". The results showed that most of the immunoglobulin A (IgA) counts in infants under 3 yr wi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197906000-00002
更新日期:1979-06-01 00:00:00
abstract::We studied the effects of anemia and ouabain administration on cardiac function, oxygen physiology, and blood catecholamine levels in nine newborn lambs. We measured oxygen consumption continuously, along with traditional hemodynamic variables. Oxygen transport was calculated. Following baseline measurements, lambs we...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198705000-00005
更新日期:1987-05-01 00:00:00
abstract::The auditory brainstem response (ABR) was monitored during infusion of bilirubin in six ventilated newborn rhesus monkeys (138-145 d gestation) while acute changes in pH were produced by varying inspired CO2. Prolonged respiratory acidosis without bilirubin infusion produced minimal changes in the ABR (one animal). CO...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199307000-00002
更新日期:1993-07-01 00:00:00
abstract::The sheep has served as an informative animal model for investigation of human fetal and newborn erythropoiesis and red blood cell (RBC) kinetics. We previously validated the permanent label (14C)cyanate for measuring red cell volume (RCV) in sheep. Here, we validate biotin labeling of RBCs as a nonradioactive method ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318183f119
更新日期:2008-11-01 00:00:00
abstract::Infection with human immunodeficiency virus (HIV) causes a progressive immunodeficiency, often manifested in adults by the development of infections with relatively rare, opportunistic organisms. However, in HIV-infected children, recurrent serious infections with common encapsulated bacteria, as well as recurrent min...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验,评审
doi:10.1203/00006450-199305001-00464
更新日期:1993-01-01 00:00:00
abstract:BACKGROUND:Oral propranolol, a nonselective β-blocker, is able to reduce the progression of retinopathy of prematurity in newborns, but it appeared unsafe. This study aimed to find, in rabbits, a propranolol eye drop concentration able to induce lower plasma but higher retinal concentrations than those obtained after o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.108
更新日期:2014-10-01 00:00:00
abstract::Hirschsprung's disease, affecting one in 5000 live newborns, is the most common cause of neonatal intestinal obstruction. The obstruction or, later in life, constipation arises from the lack of enteric ganglia in the hindgut, thus resulting in poor coordination of peristalsis. Mutations in Hirschsprung patients have s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199905010-00018
更新日期:1999-05-01 00:00:00
abstract::We studied choline uptake by slices of adult and 10-day-old rat intestine which were exposed on their mucosal surface to radiolabeled choline. Both neonatal and adult intestine transported choline. Choline uptake was observed in duodenum, jejunum, ileum, and colon of the adult rat. In the small intestine, choline upta...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198608000-00014
更新日期:1986-08-01 00:00:00
abstract::A technique is described in which paper chromatoelectrophoresis is used to measure the bilirubin binding capacity of albumin in serum. It is equivalent to the Sephadex G-25 method and its results are highly reproducible. From a single pool of serum, multiple determinations indicated the mean bilirubin binding capacity...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197707000-00006
更新日期:1977-07-01 00:00:00
abstract::The auditory brainstem response (ABR) was monitored in nine infant rhesus monkeys during the intravenous infusion of 50-168 mg/kg of unconjugated bilirubin. Sulfisoxazole (200 mg/kg) was sometimes given near the end of or just before the bilirubin infusion if no obvious ABR change had yet occurred. Five of the animals...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00005
更新日期:1986-06-01 00:00:00
abstract:BACKGROUND:Irisin is a myokine secreted by skeletal muscle during physical activity. Irisin treatment increased cortical bone mineral density (BMD) in young healthy mice and restored bone and muscle mass loss in a mouse model of disuse-induced osteoporosis and muscular atrophy. In humans, Irisin was positively correlat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0278-y
更新日期:2019-03-01 00:00:00
abstract::Isolated TSH deficiency leading to hypothyroidism seems to be a rare condition, escaping the diagnosis by neonatal screening programs, which are based on the primary determination of TSH. This is the first report of a case with an autosomal recessive TSH defect caused by a homozygous mutation of the betaTSH gene that ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199908000-00007
更新日期:1999-08-01 00:00:00
abstract::Alterations in the pancreatic secretion of fluid and of enzymes in response to either pilocarpine (15 mg/kg) or an octapeptide of cholecystokinin (0.1 microgram/kg) have been found in rats that received daily injections of reserpine (0.5 mg/kg) for 7 days. During a 3-hr secretory period, significant reductions in the ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198109000-00015
更新日期:1981-09-01 00:00:00
abstract::Several hours after an hypoxic-ischemic injury to the developing brain, hyperemia, then seizures, edema, and infarction can develop. The roles of nitric oxide (NO) synthesis and excitotoxin accumulation during these later phases of injury are not known. The time course of extracellular levels of amino acids within the...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199605000-00008
更新日期:1996-05-01 00:00:00
abstract::An assessment of pulsatile secretion of luteinizing hormone (LH), measured by both immunoassay (I-LH) and rat interstitial cell testosterone production bioassay (B-LH), as well as of follicle-stimulating hormone and glycoprotein hormone alpha-subunit was carried out in seven normal prepubertal and six normal premenarc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198704000-00018
更新日期:1987-04-01 00:00:00
abstract::Rats were fed a 50% galactose diet during pregnancy and nursing, and the testes were later examined and hormone levels determined in male offspring. Exposure to galactose for various periods during pregnancy, throughout the entire gestation, or postnatally to nursing mother until pups were 5 wk of age produced no sign...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198404000-00008
更新日期:1984-04-01 00:00:00
abstract:BACKGROUND:Neonatal haemorrhaging is often co-observed with thrombocytopenia; however, no evidence of a causal relationship with low platelet count has been reported. Regardless, the administration of a platelet transfusion is often based upon this parameter. Accurate measurement of platelet function in small volumes o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0316-9
更新日期:2019-05-01 00:00:00
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
更新日期:1984-12-01 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:BACKGROUND:Pediatric myocarditis is a rare disease with substantial mortality. Little is known regarding its prognostic factors. We hypothesize that certain comorbidities and procedural needs may increase risks of poor outcomes. This study aims to identify prognostic factors for pediatric myocarditis. METHODS:The nati...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-01268-7
更新日期:2020-11-18 00:00:00