243 results match your criteria Biochemical and molecular medicine[Journal]


Sequence analysis of the 3' hypoxia-responsive element of the human erythropoietin gene in patients with erythrocytosis.

Biochem Mol Med 1997 Oct;62(1):132-4

Institute of Clinical Sciences, The Queen's University of Belfast, Grosvenor Road, Belfast, BT12 6BA, Northern Ireland.

Erythrocytosis arises from a variety of pathogenic mechanisms. We sequenced a 256-bp region 3' to the erythropoietin (Epo) gene which included a 24- to 50-bp minimal hypoxia-responsive element spanning HIF-1- and HNF-4-binding sites in 12 patients with erythrocytosis and 4 normal subjects. Four polymorphisms were found, none of which affected the HIF-1-binding site, although one polymorphism was present in the HNF-4 consensus region. Read More

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October 1997

Oxidation of bilirubin by rat brain mitochondrial membranes-genetic variability.

Biochem Mol Med 1997 Oct;62(1):128-31

Department of Anesthesia and Critical Care Medicine, University of Pittsburgh, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania 15213-2583, USA.

Bilirubin is oxidized by brain mitochondrial membranes at a rate which may contribute significantly to clearance of bilirubin from brain. Different strains of congenitally jaundiced rats (Gunn rats) vary widely as far as the mortality rate of the homozygous (jaundiced) pups. Because the ability to oxidize bilirubin in brain may protect against toxicity, we hypothesized that the ability to oxidize bilirubin would be lower in Gunn rat strains (ACI/N-j) with a high mortality rate in the homozygous pups. Read More

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October 1997

Retrovirus vectors displaying the IgG-binding domain of protein A.

Authors:
K Ohno D Meruelo

Biochem Mol Med 1997 Oct;62(1):123-7

Kaplan Cancer Center, New York University Medical Center, 560 First Avenue, New York, New York 10016, USA.

We have designed and constructed retrovirus particles displaying the IgG-binding domain of protein A. We fused the gene for the synthetic antibody-binding portion of protein A with the envelope gene of ecotropic Moloney murine leukemia virus. The fusion gene was coexpressed in ecotropic retroviral packaging cells, and retrovirus particles with IgG-binding activities were recovered. Read More

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October 1997
1 Read

Organ-specific over-sulfation of glycosaminoglycans and altered extracellular matrix in a mouse model of cystic fibrosis.

Biochem Mol Med 1997 Oct;62(1):113-22

Department of Chemical Pathology, Women's and Children's Hospital, Adelaide, South Australia, 5006, Australia.

Cystic fibrosis (CF) is a fatal inherited disease caused by the loss of function of a plasma membrane chloride channel-the cystic fibrosis transmembrane conductance regulator (CFTR). It is characterized by viscous mucous secretions which have abnormal glycosylation and sulfation. The development of a CFTR knockout mouse has allowed in vivo experiments aimed at investigating the over-sulfation phenomenon reported for CF glycoconjugates. Read More

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October 1997

Na-Li countertransport kinetics in the relatives of hypertensive patients with abnormal Na-Li countertransport activity.

Biochem Mol Med 1997 Oct;62(1):106-12

Department of Medicine (Nephrology), University of Newcastle upon Tyne, Newcastle upon Tyne, United Kingdom.

Familial factors are believed to be important in determining the high sodium-lithium countertransport activity (defined as >0.40 mmol Li/(h x l cell) at external sodium concentration of 140 mmol/L (Nae 140)) which is observed in a proportion of patients with essential hypertension. However, environmental factors such as pregnancy and dyslipidemia also affect activity. Read More

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October 1997

Tetrahydrobiopterin in the treatment of infantile hypertrophic pyloric stenosis.

Biochem Mol Med 1997 Oct;62(1):101-5

Department of Pediatrics, University of Zürich, Zürich, 8032, Switzerland.

Evidence is emerging that reduced nitric oxide production may be involved in the pathogenesis of hypertrophic pyloric stenosis. Nitric oxide synthase (NOS) requires tetrahydrobiopterin (BH4) for activity. Four infants with hypertrophic pyloric stenosis were treated with oral BH4 (10 mg/kg/day) for 2. Read More

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October 1997
2 Reads

Demonstration of the presence of cyclic inositol phosphohydrolase in human urine.

Biochem Mol Med 1997 Oct;62(1):95-100

Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama 35294, USA.

Cyclic inositol phosphohydrolase (cIPH), cleaves the cyclic bond of cyclic inositol monophosphate (cIP) to yield inositol monophosphate. In this communication, we demonstrate the presence of cIPH in human urine. cIPH was measured in the 24-h urine samples of both male and female hospital patients. Read More

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October 1997

Sulfation of chondroitin/dermatan sulfate by cystic fibrosis pancreatic duct cells is not different from control cells.

Biochem Mol Med 1997 Oct;62(1):85-94

Department of Chemical Pathology, Women's and Children's Hospital, Adelaide, South Australia, 5006.

Cystic fibrosis is associated with mutations of the cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-regulated plasma membrane chloride channel. Cystic fibrosis patients have been reported to possess elevated sulfation of glycoconjugates, which may contribute to the pathogenesis of the disease. Sulfation of glycosaminoglycans by a cystic fibrosis pancreatic adenocarcinoma cell line homozygous for DeltaF508 (CFPAC-1), a control pancreatic cell line (PANC-1), two CFPAC-1 cell lines transfected with the gene for CFTR (PLJ-CFTR-4. Read More

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October 1997

Insulinotropic action of 1,2,3-Tri(methylsuccinyl)glycerol ester.

Biochem Mol Med 1997 Oct;62(1):76-84

Laboratory of Experimental Medicine, Brussels Free University, Brussels, Belgium.

A novel ester of succinic acid, 1,2,3-tri(methylsuccinyl)glycerol ester (3SMG), was found to stimulate insulin release from rat pancreatic islets. In the presence of 7 mM d-glucose, a 10 microM concentration of 3SMG was sufficient to cause a significant increase in insulin output. The ester mimicked the effect of other nutrient secretagogues in enhancing the synthesis of islet peptides, with a preferential action on proinsulin as distinct from nonhormonal peptides, in decreasing 86Rb outflow from prelabeled islets, and in stimulating Ca2+ inflow into the islet cells. Read More

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October 1997

Changes in plasma glucose, insulin, glucagon, catecholamine, and glycogen contents in tissues during development of alloxan diabetes mellitus in rats.

Biochem Mol Med 1997 Oct;62(1):70-5

The Ohashi Hospital, Toho University, Tokyo, Japan.

Alloxan monohydrate (ALX) was given to rats (20 mg/100 g body weight) and plasma glucose (PG), immunoreactive insulin (IRI), immunoreactive glucagon (IRG), and catecholamine (CA) as well as the glycogen (G) content in the liver, muscle, and kidneys were measured. Although whether hypoglycemia was present immediately after injection was not clear, the PG level increased, with a modest peak after 2 h. The PG levels in rats receiving food 6 h after ALX injection increased substantially after 1 h and continued to increase after 24 h. Read More

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October 1997

Increased glucagon action on lactate gluconeogenesis in perfused liver of dexamethasone-treated rats.

Authors:
O Mokuda Y Sakamoto

Biochem Mol Med 1997 Oct;62(1):65-9

Third Department of Internal Medicine, Teikyo University School of Medicine, Ichihara, Chiba, 29901, Japan.

To clearly understand the hyperglycemic action of glucocorticoids, we studied the action of glucagon on lactate gluconeogenesis in the liver of rats 7 days after adrenalectomy and after treatment with 1 mg/kg dexamethasone for 7 days. The liver was isolated and cyclically perfused at 20 ml/min with 25 ml of perfusion medium containing 5 mM lactate, [U-14C]lactate, and 0-100 ng/ml glucagon. In the absence of glucagon, incorporation of [14C]lactate into glucose carbon 1 did not change significantly in the adrenalectomized rat liver (1. Read More

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October 1997

The pathogenetic role of heme in pregnancy-induced hypertension-like disease in ewes.

Biochem Mol Med 1997 Oct;62(1):58-64

Department of Pediatrics, Albert Szent-Györgyi University Medical School, Szeged, Hungary.

Toxicosis syndrome of fasting pregnant ewes has a close similarity to human preeclampsia (hypertension, albuminuria). The common etiological factor might be oxidative hemolysis and heme-induced endothelial damage. Ewes (5 starving, 5 control) at 130-135 gestational days with a 96-h fasting period followed by refeeding were used. Read More

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October 1997

Subnuclear localization of protein kinase C delta in beta cells.

Biochem Mol Med 1997 Oct;62(1):50-7

College of Veterinary Medicine, The University of Georgia, Athens, Georgia 30602, USA.

Our laboratory has previously shown that beta cells express multiple isoforms of protein kinase C (PKC) and that some isoforms are located to multiple pools within the cell, including the cytoskeletal elements. In this study we analyzed the localization of the delta, epsilon, zeta, beta, and alpha isoforms of PKC to the nucleus. Nuclei were isolated from insulinoma beta cells and fractionated by centrifugation to give the nuclear soluble fraction, nuclear membrane fraction, and the insoluble matrix. Read More

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October 1997

Cholesteryl ester storage disease: relationship between molecular defects and in situ activity of lysosomal acid lipase.

Biochem Mol Med 1997 Oct;62(1):42-9

"Maladies Métaboliques,", INSERM U. 466, CHU Rangueil, F-31403 Toulouse, France.

The molecular defects in the LIPA gene encoding the lysosomal acid lipase (LAL) were investigated in two unrelated patients affected with cholesteryl ester storage disease (CESD), an autosomal recessive disorder associated with LAL-deficient activity. In cell lysates from both patients there was a severely reduced LAL activity. In a female patient, nucleotide sequencing of amplified LAL genomic DNA or reverse-transcribed mRNA demonstrated that she was a compound heterozygote for two previously reported mutations, a G --> A transition at position -1 of the exon 8 splice donor site, resulting in skipping of the complete exon 8, and a C923 --> T substitution leading to the replacement of His274 to Tyr. Read More

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October 1997

Enhanced resistance of adriamycin-treated MCR-5 lung fibroblasts by increased intracellular glutathione peroxidase and extracellular antioxidants.

Biochem Mol Med 1997 Oct;62(1):36-41

Faculty of Pharmacy, University of Catania, V.le A. Doria, 6, Catania, 95125, Italy.

Considerable evidence indicates that reactive oxygen species play an etiological role in both cardiotoxicity and the skin necrosis induced by adriamycin (ADM). An increase in glutathione peroxidase activity on addition of selenium to cultured MCR-5 lung fibroblasts was observed; this increase was accompanied by enhanced cellular resistance to ADM toxicity. Moreover, the presence of exogenous antioxidant systems, such as superoxide dismutase, catalase, vitamin E, dimethylsulfoxide, and desferroxamine, an iron chelating agent, resulted in significant protection from ADM-mediated damage. Read More

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October 1997

Phenotypic comparison of an osteogenesis imperfecta type IV proband with a de novo alpha2(I) Gly922 --> Ser substitution in type I collagen and an unrelated patient with an identical mutation.

Biochem Mol Med 1997 Oct;62(1):26-35

Department of Biochemistry "A. Castellani", University of Pavia, Pavia, Italy.

We examined the type I collagen synthesized by cultured dermal fibroblasts from a patient affected with osteogenesis imperfecta (OI) type IV. Both normal and abnormal trimers were produced. The mutant collagen molecules were excessively modified intracellularly, had a melting temperature 4 degrees C lower than the control, were secreted at a reduced rate, and underwent delayed processing to mature alpha chains. Read More

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October 1997

A common 844INS68 insertion variant in the cystathionine beta-synthase gene.

Biochem Mol Med 1997 Oct;62(1):23-5

Department of Pediatrics, University Hospital Nijmegen, Nijmegen, 6500 HB, The Netherlands.

Mildly elevated plasma homocysteine has been shown to be associated with an elevated risk for cardiovascular disease. In this study, we analyzed the frequency of a common 844ins68 insertion variant in the cystathionine beta-synthase gene (CBS) in patients with arterial occlusive disease and in controls and assessed the association between the insertion variant and plasma homocysteine concentrations. The insertion variant was equally distributed between both study groups. Read More

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October 1997

Therapeutic potential and mechanism of action of oligonucleotides and ribozymes.

Biochem Mol Med 1997 Oct;62(1):11-22

Cellular and Structural Biology, The University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, Texas 78284-7762, USA.

Specific inactivation of gene expression is an attractive approach for rational drug design to combat degenerative diseases and infectious agents. Oligonucleotide-directed triple-helix formation at cis-acting elements of gene promoters, short oligonucleotides containing base sequences that are complementary to the messenger RNA (antisense oligos), and RNA enzymes (ribozymes) that specifically cleave messenger RNA molecules are currently being used both as experimental tools and as therapeutic agents. Mechanisms of action of various oligonucleotide-based drugs, recent developments in the drug-delivery approaches, and future potentials are discussed in this review. Read More

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October 1997

The old and the new in p53 functional regulation.

Biochem Mol Med 1997 Oct;62(1):3-10

Laboratory of Molecular Biology at the Institute of General Pathology, University of Florence , Viale Morgagni 50, Florence, 50134, Italy.

The gene termed p53 is one of the most extensively studied for the past 18 years and the amount of literature published on this gene reflects its relevance in the field of molecular oncology; thus, loss or mutation of this oncosuppressor gene is probably the molecular lesion most frequently observed in human tumors. The aim of this minireview is to report, discuss, and interpret some recent observations on this topic: (I) The relationship with the Ataxia-Telangectasia gene and with the signaling enzyme phosphatidylinositol 3-kinase (PI3K). (II) The relationship between DNA damage, p53, and sensitivity to anticancer therapies. Read More

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October 1997

Genomic DNA from mice: a comparison of recovery methods and tissue sources.

Biochem Mol Med 1997 Dec;62(2):197-202

Department of Psychiatry, Indiana University School of Medicine, Indianapolis 46202, USA.

Our aim is to identify an extraction method and the source of mouse tissue(s) that could allow a high-resolution genomic scan from a living mouse. We compared and optimized two methods for yield, purity of DNA, and their use in the polymerase chain reaction (PCR) of DNA extracted from different mouse tissues. In addition to whole blood, tissue samples from the brain, liver, testis, and tail were included in this study. Read More

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December 1997

Monoamine oxidase and semicarbazide-sensitive amine oxidase activities in isolated cardiomyocytes of spontaneously hypertensive rats.

Biochem Mol Med 1997 Dec;62(2):188-96

Department of Pharmacology, University of Florence, Italy.

In the isolated cardiomyocytes of spontaneously hypertensive rats (SHR, 3 months old) MAO A and B activities were significantly increased compared to the myocytes in the hearts of age-matched Wistar-Kyoto rats. This increase was not associated with cardiac hypertrophy in these young animals, but might represent an early event in the development of hypertrophy. A semicarbazide-sensitive amine oxidase (SSAO) activity was found in cardiomyocytes. Read More

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December 1997
5 Reads

Preimplantation diagnosis of single gene disorders by two-step oocyte genetic analysis using first and second polar body.

Biochem Mol Med 1997 Dec;62(2):182-7

Reproductive Genetics Institute, Illinois Masonic Medical Center, Chicago 60657, USA.

Previous work on preimplantation genetic diagnosis (PGD) of single gene disorders by the first polar body (IPB) analysis has demonstrated that the genotype of a considerable number of embryos resulting from heterozygous oocytes cannot be predicted without testing their second PB (IIPB). To overcome this limitation we introduce a two-step DNA analysis of oocytes using both IPB and IIPB to identify hemizygous mutation-free oocytes following the second meiotic division. In the application of the approach to PGD of cystic fibrosis (CF) Delta F-508 mutation, sickle cell disease, and hemophilia B, 80 oocytes were studied by both PBs, resulting in the identification and transfer of 32 homozygous normal embryos. Read More

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December 1997

A common deletion mutation in European patients with Sjögren-Larsson syndrome.

Biochem Mol Med 1997 Dec;62(2):178-81

Department of Pediatrics, Medical College of Virginia, Virginia Commonwealth University, Richmond 23298, USA.

Sjögren-Larsson syndrome (SLS) is an inherited neurocutaneous disorder characterized by ichthyosis, mental retardation, spasticity, and deficient activity of fatty aldehyde dehydrogenase (FALDH). We identified a frequent FALDH mutation in exon 9 among SLS probands of European descent. This mutation is a 2-bp deletion of nucleotides GA 1297-1298 and results in premature termination of translation at codon 435 along with substitution of Arg and Cys for Glu433 and Gly434 respectively. Read More

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December 1997

Enzyme-linked immunosorbent assay of autoantibodies against mitochondrial glycerophosphate dehydrogenase in insulin-dependent and non-insulin-dependent diabetic subjects.

Biochem Mol Med 1997 Dec;62(2):172-7

Endocrinology Unit, Hospital Clinic, Barcelona, Spain.

The mitochondrial enzyme FAD-linked glycerophosphate dehydrogenase (mGDH) plays a key role in the recognition of glucose as a stimulus for insulin release from the pancreatic islet B-cell. In the present study, an ELISA procedure was used for the measurement of mGDH antibodies in both insulin-dependent (IDDM) and non-insulin-dependent (NIDDM) diabetic patients. Positive readings, exceeding the upper limit of the normal range, were recorded in 7 out of 12 IDDM patients, as distinct (P < 0. Read More

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December 1997

Qualitative and quantitative changes in skeletal muscle mtDNA and expression of mitochondrial-encoded genes in the human aging process.

Biochem Mol Med 1997 Dec;62(2):165-71

Department of Internal Medicine, Hospital Clinic, University of Barcelona, Spain.

It has been widely postulated that age-dependent changes in the mitochondrial genetic system may contribute to the human aging process. We recently reported unchanged specific activities of mitochondrial respiratory chain enzymes and a decrease in oxidation capacity of different substrates with aging, due, in part, to some confounding variables such as physical activity or tobacco consumption. The present study deals with age-related changes in muscle mtDNA structure and its biogenesis in humans. Read More

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December 1997

Analysis of the 5' flanking region of the human galactocerebrosidase (GALC) gene.

Biochem Mol Med 1997 Dec;62(2):159-64

Department of Medicine (Medical Genetics), Jefferson Medical College, Philadelphia, Pennsylvania 19107, USA.

Galactocerebrosidase (GALC) is the lysosomal enzyme deficient in human and certain animal species with globoid cell leukodystrophy (GLD) or Krabbe disease. It catalyzes the hydrolysis of specific galactolipids including galactosylceramide and psychosine. The GALC protein is found in very low amounts in all tissues, which delayed its purification and the subsequent cloning of its cDNA and gene. Read More

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December 1997

Hyperandrogenism and manifesting heterozygotes for 21-hydroxylase deficiency.

Biochem Mol Med 1997 Dec;62(2):151-8

Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pennsylvania 15213, USA.

Premature adrenarche and functional adolescent androgen excess are common disorders which may evolve into polycystic ovary syndrome (PCOS). In all three disorders, ACTH-stimulated 17-hydroxyprogesterone concentrations are often somewhat elevated. To determine the role of 21-hydroxylase (CYP21) gene mutations in these disorders, we performed molecular genotype analysis on 48 children and adolescents referred for evaluation of hyperandrogenic findings and diagnosed as having premature adrenarche or functional androgen excess. Read More

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December 1997

Differential effects of Wilms tumor WT1 splice variants on the insulin receptor promoter.

Biochem Mol Med 1997 Dec;62(2):139-50

UCSD/Whittier Diabetes Program, University of California, San Diego, La Jolla 92093, USA.

The Wilms tumor gene WT1 has been implicated in the early development of the kidney. Mutations in WT1 are found in a small fraction of Wilms tumor, a pediatric nephroblastoma, and Denys-Drash syndrome, characterized by genitourinary abnormalities. The WT1 gene product functions as a transcriptional repressor of growth factor-related genes. Read More

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December 1997

EDITORIAL

Authors:
McCabe

Biochem Mol Med 1997 Dec;62(2):137-8

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December 1997

Editorial

Authors:
McCabe

Biochem Mol Med 1997 Oct;62(1):1-2

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October 1997

Preliminary investigation of the use of dried-blood spots for the assessment of in utero exposure to environmental pollutants.

Biochem Mol Med 1997 Aug;61(2):236-9

Centers for Disease Control and Prevention (CDC), Atlanta, Georgia 30341-3724, USA.

We determined the concentration of dichlorodiphenyldichloroethylene (p,p'-DDE) in dried-blood spot specimens from 2-day-old infants from rural Texas who had never been breast fed. Anonymous, residual whole blood spots on filter paper, previously used for routine newborn screening procedures, were soaked in a phosphate buffer, extracted with an organic solvent, and eluted through silica gel. The concentrated eluates were analyzed by capillary gas chromatography with electron capture detection (ECD). Read More

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Asymmetrical labeling of D-glucose generated from [3(-13)C]pyruvate in rat hepatocytes.

Biochem Mol Med 1997 Aug;61(2):229-35

High Resolution NMR Centre, Free University of Brussels (VUB), Belgium.

The generation of 13C-labeled D-glucose isotopomers by rat hepatocytes incubated for 30 or 120 min in the presence of 10 mM [3-(13)C]pyruvate was assessed by 13C NMR. The amount of C1-labeled D-glucose exceeded that of C2-labeled hexose, which was itself higher than that of C3-labeled D-glucose. A comparable hierarchy was observed in the C6-C5-C4 moiety of the hexose. Read More

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Isolation and partial characterisation of insulin-mimetic inositol phosphoglycans from human liver.

Biochem Mol Med 1997 Aug;61(2):214-28

Department of Molecular Pathology, University College London Medical School, United Kingdom.

Extracts of human liver were found to contain activities which copurified and coeluted with the two major subtypes of mediators (type A and type P) isolated from insulin-stimulated rat liver. The putative type A mediator from human liver inhibited cAMP-dependent protein kinase from bovine heart, decreased phosphoenolypyruvate carboxykinase mRNA levels in rat hepatoma cells, and stimulated lipogenesis in rat adipocytes. The putative type P mediator stimulated bovine heart pyruvate dehydrogenase phosphatase. Read More

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Comparative analysis of apo(a) gene alleles: distribution of pentanucleotide repeats in position -1373 and C/T transition in position +93 among patients with myocardial infarction and a control group in St. Petersburg, Russia.

Biochem Mol Med 1997 Aug;61(2):208-13

Laboratory of Human Molecular Genetics, Petersburg Nuclear Physics Institute RAS, Gatchina, Russia.

To evaluate whether polymorphisms in the 5' region of the apolipoprotein(a) gene alter the risk for myocardial infarction, 289 Russian male patients with myocardial infarction (MI) and 284 subjects in a control group were investigated regarding the distribution of pentanucleotide repeats (PNRs) at position -1373 and a C/T transition at position +93. For detection of the C/T (+93) allele, we developed a rapid, nonisotopic method by mismatch PCR-mediated site-directed mutagenesis and restriction enzyme digestion. We observed significant differences in prevailing alleles with over eight (TTTTA) repeats among MI patients, including those with MI younger than 55 years of age. Read More

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Biochemical features of a patient with Zellweger-like syndrome with normal PTS-1 and PTS-2 peroxisomal protein import systems: a new peroxisomal disease.

Biochem Mol Med 1997 Aug;61(2):198-207

Pediatrics Department, Medical University of South Carolina, Charleston 29425, USA.

The peroxisomal disorders represent a group of inherited metabolic disorders that derive from defects of peroxisomal biogenesis and/or from dysfunction of single or multiple peroxisomal enzymes. We described earlier an 8 1/2 year-old with a history of progressive developmental delay, micronodular cirrhosis, and elevated very long chain fatty acids in plasma and skin fibroblasts. These findings were felt to be compatible with both neonatal adrenoleukodystrophy (nALD) and Zellweger syndrome (ZS). Read More

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Transcription of the amylin gene in newborn dogs.

Biochem Mol Med 1997 Aug;61(2):192-7

Department of Pediatrics, Medical College of Wisconsin, Milwauhee 53226, USA.

To understand the role of amylin, the novel pancreatic hormone, in fuel metabolism of neonatal mammals, the transcription of the amylin gene in newborn dogs was studied under different conditions, such as fasting, hyperinsulinemia, and hyper IGF-1. Our results showed (1) The amylin mRNA level decreased during a 24-h fasting period after birth, 59.1 +/- 4. Read More

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Acidic FGF regulation of hyperproliferation of fibroblasts in human autosomal dominant polycystic kidney disease.

Biochem Mol Med 1997 Aug;61(2):178-91

Department of Physiology, UMDNJ-RWJ (formerly Rutgers) Medical School, Piscataway 08854, USA.

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by cystic tubule enlargement and expansion of the interstitium associated with fibrosis. Our previous studies have analyzed the increased proliferation of cystic epithelial cells and this study examines the basis of increased proliferation of interstitial fibroblasts associated with ADPKD disease progression. ADPKD fibroblasts show phenotypic alterations in vitro, have acquired the capacity to grow in soft agar, and show an increased mitogenic response to a variety of growth factors particularly acidic FGF (aFGF). Read More

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Isolation and nucleotide sequence of canine glucose-6-phosphatase mRNA: identification of mutation in puppies with glycogen storage disease type Ia.

Biochem Mol Med 1997 Aug;61(2):168-77

Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.

Two Maltese puppies with massive hepatomegaly and failure to thrive had isolated deficient glucose-6-phosphatase (G-6-Pase) activity in liver and kidney and pathological findings compatible with GSD-Ia. To identify the mutation, we cloned G-6-Pase canine cDNA by RT-PCR with primers from the murine G-6-Pase gene sequence. The canine G-6-Pase cDNA is 2346 bp, with a 5' untranslated region of 87 bp, a coding region of 1071 bp, and a 3' untranslated region of 1185 bp. Read More

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Abnormal metabolism of mannose in families with carbohydrate-deficient glycoprotein syndrome type 1.

Biochem Mol Med 1997 Aug;61(2):161-7

Burnham Institute, La Jolla, California 92037, USA.

Patients with carbohydrate-deficient glycoprotein syndrome (CDGS) Type 1 underglycosylate many glycoproteins by failing to add entire N-linked carbohydrate chains to them. The primary defect in these patients has been reported as a > 90% deficiency in phosphomannomutase activity (PMM), the enzyme that converts mannose-6-phosphate to mannose-1-phosphate. This lesion reduces both the amount and the size of the lipid-linked oligosaccharide precursor. Read More

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Increased inducible nitric oxide synthase in skeletal muscle biopsies from patients with chronic heart failure.

Biochem Mol Med 1997 Aug;61(2):152-60

Heart-Center, University of Leipzig, Germany.

In addition to left ventricular pump failure and low cardiac output, structural and metabolic alterations of skeletal muscle are thought to contribute to exercise intolerance seen in patients with CHF. Studies using cardiac myocytes have implicated nitric oxide elaborated by inducible nitric oxide synthase (iNOS) as a potential agent associated with the genesis of dilated cardiomyopathy. The present study was designed to locate iNOS in the working skeletal muscle of patients with congestive heart failure. Read More

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An evaluation of the use of dried blood spots from newborn screening for monitoring the prevalence of cocaine use among childbearing women.

Biochem Mol Med 1997 Aug;61(2):143-51

Division of Environmental Health Laboratory Sciences, Centers for Disease Control and Prevention, Atlanta, Georgia 30333, USA.

A collaborative March of Dimes study was designed to examine the utility of dried blood spot (DBS) materials routinely collected from newborns as a source for monitoring cocaine exposure and to assess the prevalence of cocaine use among childbearing women in Georgia. We used a modified urinary radioimmunoassay (RIA) to anonymously detect the cocaine metabolite benzoylecgonine (BE) in DBSs. Extensive efforts were undertaken to assure absolute nonlinkage of BE data to any individual. Read More

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New physiological importance of two classic residual products: carbon monoxide and bilirubin.

Authors:
G Marilena

Biochem Mol Med 1997 Aug;61(2):136-42

Department of Biochemistry, University of Medicine and Pharmaceutics, Carol Davila, Bucharest, Romania.

Heme oxygenase the rate-limiting step in the degradation of heme to bilirubin, generates carbon monoxide. This gaseous molecule plays important roles in neuronal signaling and modulation of vascular tone. Additionally, carbon monoxide is involved in some pathological conditions (e. Read More

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Structure-function relationships of the PEA3 group of Ets-related transcription factors.

Biochem Mol Med 1997 Aug;61(2):127-35

UMR 319 CNRS, Institut Pasteur de Lille, France.

The PEA3 group of transcription factors belongs to the Ets family and is composed of PEA3, ERM, and ER81, which are more than 95% identical within the DNA-binding domain--the ETS domain--and which demonstrate 50% aa identity overall. We present here a review of the current knowledge of these transcription factors, which possess functional domains responsible for DNA-binding, DNA-binding inhibition, and transactivation. Recent data suggest that these factors are targets for signaling cascades, such as the Ras-dependent ones, and thus may contribute first to the nuclear response to cell stimulation and second to Ras-induced cell transformation. Read More

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Science and medicine at the millennium.

Authors:
A Kornberg

Biochem Mol Med 1997 Aug;61(2):121-6

Department of Biochemistry, Stanford University Medical Center, California 94305-5307, USA.

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August 1997
1 Read

Active form of Pseudomonas mevalonii 3-hydroxy-3-methylglutaryl coenzyme A reductase.

Biochem Mol Med 1997 Jun;61(1):114-20

Department of Biochemistry, Purdue University, West Lafayette, Indiana 47907-1153, USA.

Based on multiple gel permeation chromatographic experiments, we report a Stokes radius of 59.7 A for Pseudomonas mevalonii 3-hydroxy-3-methylglutaryl coenzyme A reductase (HMG-CoA reductase; EC 1.1. Read More

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The mechanisms of compound 48/80-induced superoxide generation mediated by A-kinase in rat peritoneal mast cells.

Biochem Mol Med 1997 Jun;61(1):107-13

Department of Pharmacology, Faculty of Pharmaceutical Sciences, Tokushimabunnri University, Tokushima, Japan.

This investigation was undertaken to clarify the mechanisms of superoxide anion (O2-) generation in rat peritoneal mast cells. Compound 48/80, a typical histamine liberator mediated by calcium influx, elicited O2- generation from the mast cells in a dose-dependent fashion. It was demonstrated by immunohistochemical study and Western blot analysis that the mast cells contained the 47-kDa phagocyte oxidase (p47phox) protein, which was one cytosolic component of the NADPH oxidase system. Read More

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Expression study of survival motor neuron gene in human fetal tissues.

Biochem Mol Med 1997 Jun;61(1):102-6

Dipartimento di Sanità Pubblica e Biologia Cellulare, Università di Tor Vergata, Rome, Italy.

In order to investigate the spinal muscular atrophy (SMA) disease processes, the expression of the survival motor neuron gene (SMN) has been analyzed in human fetal tissues using RT-PCR and in situ hybridization. These studies allowed the detection of SMN RNA in all the examined tissues, with no significant variation between different developmental stages. In particular, SMN mRNA was detected in spinal cord (dorsal and ventral portions), skeletal muscle, lung, heart, kidney, liver, and spleen. Read More

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Human seminal plasma soluble 5'-nucleotidase: regulatory aspects of the dephosphorylation of nucleoside 5'-monophosphates.

Biochem Mol Med 1997 Jun;61(1):95-101

Dipartimento di Biologia Cellulare e Molecolare, Università degli Studi di Perugia, Italy.

Human seminal plasma contains two enzyme activities both capable of dephosphorylating all nucleoside 5-monophosphates with different efficiency and specificity. Broad-spectrum soluble 5'-nucleotidase is the object of this paper which deals with the definition of the response of this enzyme to effectors, some physiological and others not naturally occurring. The enzyme did not show any product regulation as all the nucleosides tested caused a moderate effect on the hydrolysis of the substrates. Read More

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The increased skeletal muscle protein turnover of the streptozotocin diabetic rat is associated with high concentrations of branched-chain amino acids.

Biochem Mol Med 1997 Jun;61(1):87-94

Departament de Bioquímica i Biologia Molecular, Facultat de Biologia, Universitat de Barcelona, Spain.

Experimental streptozotocin-induced diabetes resulted in important changes in body weight which were associated with abnormalities in water and food intake. In addition, diabetic rats showed a clear muscle atrophy involving a decrease in both skeletal muscle size and protein content. This was accompanied by a marked loss of total carcass nitrogen. Read More

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Inhibition of transforming growth factor beta 1 induction by dietary vitamin E in unilateral ureteral obstruction in rats.

Biochem Mol Med 1997 Jun;61(1):82-6

Department of Pediatrics, Virginia Commonwealth University's Medical College of Virginia, Richmond 23298, USA.

Free radical species associated with bilateral ureteral obstruction (BUO) are considered important in the pathogenesis of the glomerular and tubulointerstitial injury in BUO rats. We seek to test the hypothesis that the use of an easily administered antioxidant, vitamin E, at sufficient plasma concentrations, can decrease this release of free oxygen radicals in kidney tissue and ameliorate the increase of the fibrogenic cytokine, transforming growth factor beta-1 (TGF beta-1). We used the unilateral ureteral obstruction (UUO) rat model, because the presence of the uninjured contralateral kidney provides a nonuremic internal milieu, in contrast to the uremic, acidotic, and hypercholesterolemic BUO model. Read More

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