Abstract:
:Galactose metabolism was studied in human ovarian tissue obtained from 14 women controls between 21 and 72 y of age, and one 21-y-old galactosemic patient with hypergonadotrophic hypogonadism. Tissue slices were incubated with 1-14C-galactose, and labeled intermediates were analyzed by anion-exchange column chromatography. Activities of enzymes related to the galactose pathway: galactokinase, transferase, epimerase, uridine diphosphoglucose (UDPGlc) and uridine diphosphogalactose pyrophosphorylases, and UDPGlc and uridine diphosphogalactose pyrophosphatases were measured in ovarian homogenates using radioisotopic, spectrophotometric, and fluorometric techniques. Incorporation of carbon label from 1-14C-galactose into various galactose and glycolytic intermediates, as well as carbon dioxide and TCA-insoluble materials was demonstrated in samples from non-galactosemic controls. In tissue from the galactosemic individual, no labeled carbon dioxide was produced and very little incorporation into TCA-insoluble material was found. Labeled galactose-1-phosphate was elevated. In normal ovarian tissue, specific activities of galactokinase, transferase, epimerase, and UDPGlc pyrophosphorylase are much higher than those found in the red cells and in testes. UDPGlc pyrophosphorylase activity is about 50 times that of transferase, suggesting that uridine nucleotide sugars have an important role in the normal development and function of the ovary. It is hypothesized that premature ovarian failure, often observed in patients with galactosemia, is due to interference with nucleotide sugar metabolism and the synthesis of galactose containing glycoproteins and glycolipids consequent to the enzymatic defect in the major pathway of galactose metabolism.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Xu YK,Ng WG,Kaufman FR,Lobo RA,Donnell GNdoi
10.1203/00006450-198902000-00015subject
Has Abstractpub_date
1989-02-01 00:00:00pages
151-5issue
2eissn
0031-3998issn
1530-0447journal_volume
25pub_type
杂志文章abstract::Bacterial sepsis is a relatively common problem in the neonatal period, particularly among prematurely delivered infants. The newborn rat has been widely used as a model for sepsis neonatorum, and in that model incomplete development of the neutrophil system has been postulated to be an important factor predisposing n...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198407000-00006
更新日期:1984-07-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198206000-00018
更新日期:1982-06-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198405000-00012
更新日期:1984-05-01 00:00:00
abstract::Epidermal growth factor (EGF) and transforming growth factor-alpha (TGF-alpha) are mitogenic to the intestinal epithelium. To further clarify their role in the developing human fetal gut, their expression was studied in fetuses at 15 to 20 wk of gestation. TGF-alpha mRNA was present throughout the gastrointestinal tra...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199305000-00012
更新日期:1993-05-01 00:00:00
abstract::The factors that mediate the postnatal fall in pulmonary vascular resistance, which is crucial for normal gas exchange, are not fully understood. The endothelium has been implicated in this phenomenon, through the release of vasorelaxant factors such as nitric oxide (NO). Human pulmonary expression of endothelial NO s...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/01.PDR.0000159575.58834.8D
更新日期:2005-05-01 00:00:00
abstract::Gluconeogenesis decreases during septic shock, but its mechanism is not well known. Tumor necrosis factor alpha (TNF-alpha), which is a key cytokine in septic shock, can increase GLUT1 gene expression and glucose uptake in muscles and fatty tissues. TNF-alpha does not alter the metabolism of hepatocytes in which GLUT2...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200104000-00018
更新日期:2001-04-01 00:00:00
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
更新日期:1987-01-01 00:00:00
abstract::Spontaneous preterm birth is a serious and common pregnancy complication associated with hormonal dysregulation, infection, inflammation, immunity, rupture of fetal membranes, stress, bleeding, and uterine distention. Heredity is 25-40% and mostly involves the maternal genome, with contribution of the fetal genome. Si...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-018-0180-z
更新日期:2019-03-01 00:00:00
abstract:BACKGROUND:Kawasaki disease (KD) is associated with expression and methylation of Fc gamma receptor genes. We characterized immunoglobulin A (IgA), IgE, IgG, and IgM receptor expression levels in KD. METHODS:Fc receptor expression levels were characterized using GeneChip Human Transcriptome Array 2.0 (HTA 2.0) with 18...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0707-y
更新日期:2019-12-09 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198510000-00006
更新日期:1985-10-01 00:00:00
abstract::The aim of this study was to produce a neonatal piglet model which, avoiding vessel ligation, exposed the whole animal to hypoxia and produced dose-dependent clinical encephalopathy and neuropathologic damage similar to that seen after birth asphyxia. Twenty-three piglets were halothane-anesthetized. Hypoxia was induc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199611000-00014
更新日期:1996-11-01 00:00:00
abstract::The risk of pulmonary hemorrhage is increased in extremely low birth weight infants treated with surfactant. The pathogenesis of this increased risk is far from clear. We tested whether exposure of cell membranes to surfactant may lead to increased membrane permeability, hypothesizing that this process may contribute ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:
更新日期:1995-01-01 00:00:00
abstract::A 10-year longitudinal study was conducted on 16 prepubescent youngsters who had undergone successful weight reduction. Their ages ranged from 2 to 10 years when the study began. In all subjects, weight reduction proceeded only by a decrease in adipose cell size (from 0.62 +/- 0.02 to 0.46 +/- 0.02 micrograms lipid pe...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198110000-00016
更新日期:1981-10-01 00:00:00
abstract::Brain injury remains one of the major unsolved problems in neonatal care, with survivors at high risk of lifelong neurodisability. It is unlikely that a single intervention can ameliorate neonatal brain injury, given the complex interaction between pathological processes, developmental trajectory, genetic susceptibili...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-019-0729-5
更新日期:2019-12-18 00:00:00
abstract::In human infants and newborn rats, white light at the intensity used to treat hyperbilirubinemia lowers serum calcium concentration. Occipital shielding or (in newborn rats) exogenous melatonin prevents this effect. Propranolol, by inhibiting melatonin synthesis, also causes hypocalcemia, which is preventable by melat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199006000-00006
更新日期:1990-06-01 00:00:00
abstract::BACKGROUNDLow birth weight is associated with childhood stunting, but equivalent associations for birth body composition (BC) remain unknown. The aim of this study was to assess associations between birth BC and height-for-age z-score (HAZ) at 2 years of age.METHODSIn a prospective cohort study, fat mass (FM) and fat-...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.59
更新日期:2017-08-01 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::Bacillus Calmette-Guerin (BCG) vaccination can protect animals from asthma, but the effect of BCG on childhood asthma prevention is controversial in humans. To verify the hypothesis that the BCG anti-asthma effect in childhood might be reversed by a respiratory virus infection, newborn BALB/c mice were divided into fi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000196368.08210.5c
更新日期:2006-02-01 00:00:00
abstract::Growth hormone (GH) regulates growth and development in the postnatal period but lacks somatotropic activity in the fetus. In contrast, the placental hormone placental lactogen (PL) stimulates amino acid transport, DNA synthesis, and somatomedin production in isolated fetal tissues, suggesting that PL may function as ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199208000-00015
更新日期:1992-08-01 00:00:00
abstract::We have recently purified autoantigen polypeptides reacting with celiac disease patient sera IgA from the extracellular noncollagenous matrix compartment of fetal lung tissue. These molecules trigger the production of different tissue antibodies, the so-called antireticulin and antiendomysium antibodies in celiac dise...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199310000-00007
更新日期:1993-10-01 00:00:00
abstract::The purpose of this project was to study the role of lipid peroxidation in oxygen-induced lung injury in the newborn lamb. It was our hypothesis that injury to the microvascular bed of the lung by oxygen would coincide with a burst of peroxidative activity and would be accompanied by an increased rate of excretion of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199012000-00014
更新日期:1990-12-01 00:00:00
abstract::Circadian rhythms of plasma renin activity (PRA) and plasma aldosterone (PA) were studied in eight healthy children, 7 to 15 years old. Blood samples were obtained at 7:00 AM, 10:00 AM, 1:00 PM, 4:00 PM, 5:00 PM, 8.00 PM, and midnight on day 1 and 4:00 AM and 7:00 AM on day 2. Children had normal activity during this ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198205000-00007
更新日期:1982-05-01 00:00:00
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::During the transition at birth to air breathing, regulation of surfactant release from alveolar type II (ATII) cells is critical. Atrial natriuretic peptide (ANP) stimulates natriuretic peptide receptor-A (NPR-A) and increases intracellular cGMP. We examined the changes in ANP and NPR-A in respiratory epithelium durin...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318163a215
更新日期:2008-03-01 00:00:00
abstract::Studies were conducted in an attempt to determine the hemolytic potential of acetylsalicylic acid (ASA) on normal intact red blood cells (RBCs). ASA (25 mg/100 ml) did not impair RBC G-6-PD, glutathione peroxidase or catalase activity, glutathione stability, or hexose monophosphate shunt activity. Hemolysis was not ob...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197809000-00009
更新日期:1978-09-01 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:BACKGROUND:Bilirubin-induced neurologic dysfunction (BIND) is a spectrum of preventable neurological sequelae in jaundiced newborns. Current total plasma bilirubin (BT) concentration thresholds for phototherapy and/or exchange transfusion poorly predict BIND. METHODS:The unbound (free) bilirubin (Bf) measured at these...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-018-0010-3
更新日期:2018-10-01 00:00:00
abstract::The diagnosis of transient renal tubular acidosis was made in a 16 months old boy. Bicarbonate titration studies revealed that the acidification defect consisted of an association of proximal and distal tubular acidosis. The effect of experimentally induced potassium depletion revealed that hyperkalemia contributed to...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197910000-00020
更新日期:1979-10-01 00:00:00
abstract::The purpose of this study was to investigate the effect of blue light phototherapy on the expression of circadian genes in peripheral blood mononuclear cells (PBMC) and plasma melatonin levels in neonates. Real-time reverse-transcriptase polymerase chain reaction (RT-PCR) was used to determine the expression of Bmal1 ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000183663.98446.05
更新日期:2005-12-01 00:00:00
abstract::Plasma selenium concentration and glutathione peroxidase activity in red blood cells were determined in subjects from different age groups. The selenium level (mean +/- SD) found in infancy (0 to 6 months) was 2 +/- 0.6 micrograms/dl, with the lowest value of 1 microgram/dl observed in a 4-month-old infant. These leve...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00021
更新日期:1986-06-01 00:00:00