Epstein-Barr virus-infected marmoset cells transfected with c-myc do not form lymphomas in mice with severe combined immunodeficiency.

Abstract:

:Epstein-Barr virus (EBV) has been associated with several malignant processes in man, most notably Burkitt lymphoma in previously healthy individuals and lesions resembling large cell non-Hodgkin lymphomas in organ transplant recipients. Mice with the severe combined immunodeficiency phenotype (SCID mice) are exquisitely susceptible to the development of EBV-associated lymphoproliferative lesions following the intraperitoneal (ip) inoculation of EBV-infected human lymphocytes. Recently, we reported that EBV-infected marmoset lymphocytes do not form lymphomas in SCID mice following ip injection, while human lymphocytes infected with the same EBV strains do. On the assumption that the EBV-infected marmoset cells were lacking a factor necessary for tumor formation, we transfected a plasmid containing c-myc into EBV-infected marmoset cells (B95-8, FF41, and W91 cells). Despite expression of the c-myc protein as determined by immunoblot and flow cytometry when probed with a monoclonal antibody, no increase over baseline lesion development was seen in SCID mice inoculated with 5 x 10(6) c-myc-expressing marmoset lymphoblastoid cells. Thus, cells that express c-myc and harbor EBV are not sufficient to form lymphomas in certain immunocompromised hosts.

journal_name

Mol Genet Metab

authors

Salimi B,O'Gorman MR,Variakojis D,Bendet M,Newman M,Poupko E,Katz BZ

doi

10.1006/mgme.1998.2708

subject

Has Abstract

pub_date

1998-07-01 00:00:00

pages

205-12

issue

3

eissn

1096-7192

issn

1096-7206

pii

S1096-7192(98)92708-1

journal_volume

64

pub_type

杂志文章
  • Long-term treatment with sodium phenylbutyrate in ornithine transcarbamylase-deficient patients.

    abstract::Ornithine transcarbamylase deficiency is a very heterogeneous urea cycle disorder resulting in hyperammonemia with various presentations from the neonatal period through adulthood. We performed a retrospective study in nine patients (four male/five female, age at diagnosis ranging from 6 days to 14 years) to evaluate ...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.2001.3156

    authors: Burlina AB,Ogier H,Korall H,Trefz FK

    更新日期:2001-04-01 00:00:00

  • S-adenosylmethionine treatment in methionine adenosyltransferase deficiency, a case report.

    abstract::Reported is a female patient with methionine adenosyltransferase I/III (MAT I/III) deficiency, who was found to have pronounced hypermethioninemia on newborn mass spectroscopy screening, and had two compound heterozygous missense mutations in the gene encoding human MAT1A protein. Hypermethioninemia persisted and her ...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2011.11.192

    authors: Furujo M,Kinoshita M,Nagao M,Kubo T

    更新日期:2012-03-01 00:00:00

  • Characterization of the human gene encoding alpha-aminoadipate aminotransferase (AADAT).

    abstract::In mammals, the conversion of alpha-aminoadipate to alpha-ketoadipate by alpha-aminoadipate aminotransferase (AADAT) is an intermediate step in lysine degradation. A gene encoding for alpha-aminoadipate aminotransferase and kynurenine aminotransferase activities had been previously identified in the rat (KAT/AadAT). W...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/s1096-7192(02)00037-9

    authors: Goh DL,Patel A,Thomas GH,Salomons GS,Schor DS,Jakobs C,Geraghty MT

    更新日期:2002-07-01 00:00:00

  • Maturational changes in ovine pulmonary metabolism of platelet-activating factor: implications for postnatal adaptation.

    abstract::We recently reported that PAF acetylhydrolase (PAF-Ah) mRNA level and PAF-Ah activity in lamb lungs are up-regulated in the immediate newborn period, thereby facilitating the fall in postnatal PAF levels as well as a fall in pulmonary vascular resistance (B. O. Ibe, F. C. Sardar, and J. U. Raj, Mol Genet Metab 69:46-5...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.2001.3253

    authors: Ibe BO,Pham HH,Kääpä P,Raj JU

    更新日期:2001-11-01 00:00:00

  • Enhanced placental cholesterol efflux by fetal HDL in Smith-Lemli-Opitz syndrome.

    abstract::Previous studies from this laboratory have shown that maternal-derived cholesterol can be effluxed from trophoblasts to fetal HDL and plasma. We had the opportunity to study for the first time the ability of HDL and plasma from a fetus with the Smith-Lemli-Opitz syndrome (SLOS) to efflux cholesterol from trophoblasts....

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2008.01.015

    authors: Jenkins KT,Merkens LS,Tubb MR,Myatt L,Davidson WS,Steiner RD,Woollett LA

    更新日期:2008-06-01 00:00:00

  • Urinary glycosaminoglycans as a potential biomarker for evaluating treatment efficacy in subjects with mucopolysaccharidoses.

    abstract::Accumulations of glycosaminoglycans (GAGs) that result from deficiencies in lysosomal hydrolases are characteristic of mucopolysaccharidoses (MPS). Enzyme replacement therapies (ERTs) are now available for several MPS diseases (MPS I, MPS II, MPS IVA, MPS VI, and MPS VII), but assessment of the efficacy of treatment c...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章,评审

    doi:10.1016/j.ymgme.2020.02.006

    authors: Kakkis E,Marsden D

    更新日期:2020-05-01 00:00:00

  • Fluxomic assay-assisted diagnosis orientation in a cohort of 11 patients with myopathic form of CPT2 deficiency.

    abstract::Carnitine palmitoyltransferase type 2 (CPT2) deficiency, a mitochondrial fatty acid oxidation disorder (MFAOD), is a cause of myopathy in its late clinical presentation. As for other MFAODs, its diagnosis may be evocated when blood acylcarnitine profile is abnormal. However, a lack of abnormalities or specificity in t...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2018.02.005

    authors: Fontaine M,Kim I,Dessein AF,Mention-Mulliez K,Dobbelaere D,Douillard C,Sole G,Schiff M,Jaussaud R,Espil-Taris C,Boutron A,Wuyts W,Acquaviva C,Vianey-Saban C,Roland D,Joncquel-Chevalier Curt M,Vamecq J

    更新日期:2018-04-01 00:00:00

  • Toll-like receptor mediated lysozyme expression in Niemann-pick disease, type C1.

    abstract::Niemann-Pick type C1 (NPC1) is a rare neurodegenerative disease. In NPC1 mouse cerebella, the antibacterial enzyme, lysozyme (Lyz2), is significantly increased in multiple cell types. Due to its possible role in toxic fibril deposition, we confirmed Lyz2 overexpression in culture in different control and NPC1 cell typ...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2020.10.009

    authors: Cougnoux A,Yerger JC,Fellmeth M,Serra-Vinardell J,Wassif CA,Cawley NX,Porter FD

    更新日期:2020-11-01 00:00:00

  • Bacterial species identification after DNA amplification with a universal primer pair.

    abstract::The diagnosis of bacterial infections can be difficult and time consuming. Rapid and reliable molecular triage of potentially infected patients, particularly the young and the elderly, would prevent unnecessary hospitalizations, reduce associated medical costs, and improve the quality of care. Polymerase chain reactio...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.1998.2795

    authors: McCabe KM,Zhang YH,Huang BL,Wagar EA,McCabe ER

    更新日期:1999-03-01 00:00:00

  • A phase I/II clinical trial of enzyme replacement therapy in mucopolysaccharidosis II (Hunter syndrome).

    abstract:OBJECTIVE:To evaluate the safety and explore the efficacy of idursulfase (recombinant human iduronate-2-sulfatase) treatment for mucopolysaccharidosis II (MPS II). STUDY DESIGN:Twelve patients were enrolled into a randomized, double-blind, placebo-controlled trial for 24 weeks followed by an open-label extension study...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章,随机对照试验

    doi:10.1016/j.ymgme.2006.09.001

    authors: Muenzer J,Gucsavas-Calikoglu M,McCandless SE,Schuetz TJ,Kimura A

    更新日期:2007-03-01 00:00:00

  • Uncovering microdeletions in patients with severe Glut-1 deficiency syndrome using SNP oligonucleotide microarray analysis.

    abstract::Glut-1 facilitates the diffusion of glucose across the blood-brain barrier and is responsible for glucose entry into the brain. Impaired glucose transport across the blood-brain barrier results in Glut-1 deficiency syndrome (Glut-1 DS, OMIM 606777), characterized in its most severe form by infantile seizures, developm...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2010.03.007

    authors: Levy B,Wang D,Ullner PM,Engelstad K,Yang H,Nahum O,Chung WK,De Vivo DC

    更新日期:2010-06-01 00:00:00

  • Evidence for a short-chain carnitine-acylcarnitine translocase in mitochondria specifically related to the metabolism of branched-chain amino acids.

    abstract::Carnitine-acylcarnitine translocase (CATR) deficiency is a severe defect in fatty acid oxidation which presents early in life most frequently with hypoglycemia, hyperammonemia, and severe cardiac abnormalities. CATR exchanges acylcarnitines of various chain lengths for free carnitine across the mitochondrial membrane....

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.1999.2950

    authors: Roe DS,Roe CR,Brivet M,Sweetman L

    更新日期:2000-01-01 00:00:00

  • A novel mutation (M310L) in the thyroid hormone receptor beta causing resistance to thyroid hormone in a Brazilian kindred and a neonate.

    abstract::Resistance to thyroid hormone (RTH) is an inherited syndrome of reduced tissue responsiveness to thyroid hormone (T3) caused by mutations in the thyroid hormone receptor beta (TRbeta). The index patient of the family reported here, a 17-year-old woman, came to medical attention because of a diffuse goiter, short statu...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.2000.3088

    authors: Furlanetto TW,Kopp P,Peccin S,Gu WX,Jameson JL

    更新日期:2000-11-01 00:00:00

  • Rare Saposin A deficiency: Novel variant and psychosine analysis.

    abstract::Saposin A is a post-translation product of the prosaposin (PSAP) gene that serves as an activator protein of the galactocerebrosidase (GALC) enzyme, and is necessary for the degradation of certain glycosphingolipids. Deficiency of saposin A leads to a clinical picture identical to that of early-infantile Krabbe diseas...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2019.08.001

    authors: Calderwood L,Wenger DA,Matern D,Dahmoush H,Watiker V,Lee C

    更新日期:2020-02-01 00:00:00

  • Absence of α-galactosidase cross-correction in Fabry heterozygote cultured skin fibroblasts.

    abstract::Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from deficiency of α-galactosidase A (GLA). Traditionally, heterozygotes were considered asymptomatic carriers of FD, but it is now apparent that the asymptomatic female carrier is the exception and most heterozygotes suffer significant multisystem...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2014.11.005

    authors: Fuller M,Mellett N,Hein LK,Brooks DA,Meikle PJ

    更新日期:2015-02-01 00:00:00

  • Incidence of maple syrup urine disease in Portugal.

    abstract::Maple syrup urine disease is an autosomal recessive disorder of branched-chain amino acids metabolism with a worldwide frequency of 1/185,000 live newborns. In Portugal, the incidence of the disease has not been assessed. Based on the review of the cases diagnosed by tandem mass spectrometry an incidence of 1/86,800 l...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2010.04.007

    authors: Quental S,Vilarinho L,Martins E,Teles EL,Rodrigues E,Diogo L,Garcia P,Eusébio F,Gaspar A,Sequeira S,Amorim A,Prata MJ

    更新日期:2010-08-01 00:00:00

  • 11q13 allelotype analysis in 27 northern American MEN1 kindreds identifies two distinct founder chromosomes.

    abstract::We analyzed constitutional and tumor DNA from 27 MEN1 kindreds not known to be related to each other. Disease allele haplotypes were constructed for each pedigree based on shared alleles from two or more affected members and from determination of allelic loss patterns in their tumors. Analysis of disease allele haplot...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.1997.2649

    authors: Emmert-Buck MR,Debelenko LV,Agarwal S,Kester MB,Manickam P,Zhuang Z,Guru SC,Olufemi SE,Burns AL,Chandrasekharappa SC,Lubensky IA,Liotta LA,Skarulis MC,Spiegel AM,Marx SJ,Collins FS

    更新日期:1998-02-01 00:00:00

  • A new diagnostic assay for glycogen storage disease type II in mixed leukocytes.

    abstract::We have established a new method for the enzymatic diagnosis of glycogen storage disease type II (Pompe disease or acid maltase deficiency) using mixed leukocytes. The method employs glycogen and 4-methylumbelliferyl-alpha-D-glucopyranoside (4MU-alphaGlc) as substrates for measuring the lysosomal acid alpha-glucosidas...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2005.10.016

    authors: Okumiya T,Keulemans JL,Kroos MA,Van der Beek NM,Boer MA,Takeuchi H,Van Diggelen OP,Reuser AJ

    更新日期:2006-05-01 00:00:00

  • Homozygous variegate porphyria in South Africa: genotypic analysis in two cases.

    abstract::Variegate porphyria is an autosomal dominant disorder of heme metabolism which results from decreased activity of the enzyme protoporphyrinogen oxidase. Clinically, the disease manifests postpubertally and is characterized by photocutaneous sensitivity and/or acute neurovisceral crises. However, in homozygous variegat...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.2000.2975

    authors: Corrigall AV,Hift RJ,Davids LM,Hancock V,Meissner D,Kirsch RE,Meissner PN

    更新日期:2000-04-01 00:00:00

  • Mechanism of biotin responsiveness in biotin-responsive multiple carboxylase deficiency.

    abstract::Holocarboxylase synthetase (HCS) catalyses the biotinylation of the four biotin-dependent carboxylases found in humans. A deficiency in HCS results in biotin-responsive multiple carboxylase deficiency. We have evaluated the biotin responsiveness associated with six missense mutations previously identified in affected ...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.1998.2785

    authors: Dupuis L,Campeau E,Leclerc D,Gravel RA

    更新日期:1999-02-01 00:00:00

  • Gaucher disease and parkinsonism, a molecular link theory.

    abstract::Mutant GBA was found recently to be the most prevalent risk factor for familial parkinsonism. The two diseases do not share common symptoms and there is no direct pathway to explain the mechanism by which GBA mutations can confer the risk. Increased burden on the degradative pathway caused by defective glucocerebrosid...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2010.08.004

    authors: Goldin E

    更新日期:2010-12-01 00:00:00

  • Transfection screening for defects in the PCCA and PCCB genes encoding propionyl-CoA carboxylase subunits.

    abstract::Propionic acidemia can result from mutations in the PCCA or PCCB genes encoding the alpha and beta subunits, respectively, of propionyl-CoA carboxylase. We have developed a method based on complementation of the enzyme defect using a lipid-mediated transient transfection of the normal human PCCA or PCCB cDNA into prim...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1006/mgme.2001.3296

    authors: Rodriguez-Pombo P,Pérez-Cerdá C,Desviat LR,Pérez B,Ugarte M,Rodríguez-Pombo P

    更新日期:2002-03-01 00:00:00

  • Acid sphingomyelinase (Asm) deficiency patients in The Netherlands and Belgium: disease spectrum and natural course in attenuated patients.

    abstract::Niemann-Pick disease (NPD) is a neurovisceral lysosomal storage disorder caused by acid sphingomyelinase (ASM) deficiency, which can be categorized as either Niemann-Pick disease type A [NPD-A], with progressive neurological disease and death in early childhood, or as Niemann-Pick disease type B [NPD-B], with a more v...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2012.06.015

    authors: Hollak CE,de Sonnaville ES,Cassiman D,Linthorst GE,Groener JE,Morava E,Wevers RA,Mannens M,Aerts JM,Meersseman W,Akkerman E,Niezen-Koning KE,Mulder MF,Visser G,Wijburg FA,Lefeber D,Poorthuis BJ

    更新日期:2012-11-01 00:00:00

  • Sensitive quantification of mosaicism using high density SNP arrays and the cumulative distribution function.

    abstract::Medicine is rapidly applying exome and genome sequencing to the diagnosis and management of human disease. Somatic mosaicism, however, is not readily detectable by these means, and yet it accounts for a significant portion of undiagnosed disease. We present a rapid and sensitive method, the Continuous Distribution Fun...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2011.12.015

    authors: Markello TC,Carlson-Donohoe H,Sincan M,Adams D,Bodine DM,Farrar JE,Vlachos A,Lipton JM,Auerbach AD,Ostrander EA,Chandrasekharappa SC,Boerkoel CF,Gahl WA

    更新日期:2012-04-01 00:00:00

  • Pyrimethamine increases β-hexosaminidase A activity in patients with Late Onset Tay Sachs.

    abstract:OBJECTIVE:To assess whether or not pyrimethamine (PMT) can be used to enhance β-hexosaminidase A activity (HexA) in subjects with Late Onset Tay Sachs (LOTS), we studied the effect of incremental doses of PMT in vivo in 9 LOTS patients carrying the αG269S/c.1278insTACT mutations. METHODS:PMT treatment was initiated at...

    journal_title:Molecular genetics and metabolism

    pub_type: 临床试验,杂志文章

    doi:10.1016/j.ymgme.2010.11.163

    authors: Osher E,Fattal-Valevski A,Sagie L,Urshanski N,Amir-Levi Y,Katzburg S,Peleg L,Lerman-Sagie T,Zimran A,Elstein D,Navon R,Stern N,Valevski A

    更新日期:2011-03-01 00:00:00

  • Glycosaminoglycan levels in dried blood spots of patients with mucopolysaccharidoses and mucolipidoses.

    abstract::Mucopolysaccharidoses (MPSs) and mucolipidoses (ML) are groups of lysosomal storage disorders in which lysosomal hydrolases are deficient leading to accumulation of undegraded glycosaminoglycans (GAGs), throughout the body, subsequently resulting in progressive damage to multiple tissues and organs. Assays using tande...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2016.12.010

    authors: Kubaski F,Suzuki Y,Orii K,Giugliani R,Church HJ,Mason RW,Dũng VC,Ngoc CT,Yamaguchi S,Kobayashi H,Girisha KM,Fukao T,Orii T,Tomatsu S

    更新日期:2017-03-01 00:00:00

  • A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate.

    abstract::We determined the ability of self-complementary adeno-associated virus (scAAV) vectors to deliver and express the pyruvate dehydrogenase E1alpha subunit gene (PDHA1) in primary cultures of skin fibroblasts from 3 patients with defined mutations in PHDA1 and 3 healthy subjects. Cells were transduced with scAAV vectors ...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2007.10.131

    authors: Han Z,Berendzen K,Zhong L,Surolia I,Chouthai N,Zhao W,Maina N,Srivastava A,Stacpoole PW

    更新日期:2008-04-01 00:00:00

  • Congenic mapping and genotyping of the tetrahydrobiopterin-deficient hph-1 mouse.

    abstract::The hph-1 ENU-mutant mouse provides a model of tetrahydrobiopterin deficiency for studying hyperphenylalaninaemia, dopa-response dystonia, and vascular dysfunction. We have successively localized the hph-1 mutation to a congenic interval of 1.6-2.8 Mb, containing the GCH gene encoding GTP cyclohydrolase I (GTP-CH I). ...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2004.04.006

    authors: Khoo JP,Nicoli T,Alp NJ,Fullerton J,Flint J,Channon KM

    更新日期:2004-07-01 00:00:00

  • The MMACHC proteome: hallmarks of functional cobalamin deficiency in humans.

    abstract::Cobalamin (Cbl, B(12)) is an essential micronutrient required to fulfill the enzymatic reactions of cytosolic methylcobalamin-dependent methionine synthase and mitochondrial adenosylcobalamin-dependent methylmalonyl-CoA mutase. Mutations in the MMACHC gene (cblC complementation group) disrupt processing of the upper-a...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2011.03.008

    authors: Hannibal L,DiBello PM,Yu M,Miller A,Wang S,Willard B,Rosenblatt DS,Jacobsen DW

    更新日期:2011-07-01 00:00:00

  • Rapid diagnosis of late-onset Pompe disease by fluorometric assay of alpha-glucosidase activities in dried blood spots.

    abstract::The enzymatic defect in Pompe disease is insufficient lysosomal acid alpha-glucosidase (GAA) activity which leads to lysosomal glycogen accumulation. We recently introduced a simple and reliable method to measure GAA activity in dried blood spots using Acarbose, a highly selective alpha-glucosidase inhibitor, to elimi...

    journal_title:Molecular genetics and metabolism

    pub_type: 杂志文章

    doi:10.1016/j.ymgme.2006.12.006

    authors: Kallwass H,Carr C,Gerrein J,Titlow M,Pomponio R,Bali D,Dai J,Kishnani P,Skrinar A,Corzo D,Keutzer J

    更新日期:2007-04-01 00:00:00