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La ricerca find articoli where soggetti phrase all words 'BETA-GLUCURONIDASE DEFICIENCY' sort by level,fasc_key/DESCEND, pagina_ini_num/ASCEND ha restituito 74 riferimenti
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    1. Kamata, Y; Okuyama, T; Kosuga, M; O'hira, A; Kanaji, A; Sasaki, K; Yamada, M; Azuma, N
      Adenovirus-mediated gene therapy for corneal clouding in mice with mucopolysaccharidosis type VII

      MOLECULAR THERAPY
    2. Frisella, WA; O'Connor, LH; Vogler, CA; Roberts, M; Walkley, S; Levy, B; Daly, TM; Sands, MS
      Intracranial injection of recombinant adeno-associated virus improves cognitive function in a murine model of mucopolysaccharidosis type VII

      MOLECULAR THERAPY
    3. Kosuga, M; Sasaki, K; Tanabe, A; Li, XK; Okawa, H; Ogino, I; Okuda, O; Arai, H; Sakuragawa, N; Kamata, Y; Azuma, N; Suzuki, S; Yamada, M; Okuyama, T
      Engraftment of genetically engineered amniotic epithelial cells corrects lysosomal storage in multiple areas of the brain in mucopolysaccharidosis type VII mice

      MOLECULAR THERAPY
    4. Vogler, C; Barker, J; Sands, MS; Levy, B; Galvin, N; Sly, WS
      Murine mucopolysaccharidosis VII: Impact of therapies on the phenotype, clinical course, and pathology in a model of a lysosomal storage disease

      PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
    5. Heuer, GG; Skorupa, AF; Alur, RKP; Jiang, KL; Wolfe, JH
      Accumulation of abnormal amounts of glycosaminoglycans in murine mucopolysaccharidosis type VII neural progenitor cells does not alter the growth rate or efficiency of differentiation into neurons

      MOLECULAR AND CELLULAR NEUROSCIENCE
    6. Van Buggenhout, GJCM; Trijbels, JMF; Wevers, R; Trommelen, JCM; Hamel, BCJ; Brunner, HG; Fryns, JP
      Metabolic studies in older mentally retarded patients: Significance of metabolic testing and correlation with the clinical phenotype

      GENETIC COUNSELING
    7. Daly, TM; Ohlemiller, KK; Roberts, MS; Vogler, CA; Sands, MS
      Prevention of systemic clinical disease in MPS VII mice following AAV-mediated neonatal gene transfer

      GENE THERAPY
    8. Kosuga, M; Takahashi, S; Tanabe, A; Fujino, M; Li, XK; Suzuki, S; Yamada, M; Kakishita, K; Ono, F; Sakuragawa, N; Okuyama, T
      Widespread distribution of adenovirus-transduced monkey amniotic epithelial cells after local intracerebral injection: Implication for cell-mediated therapy for lysosome storage disorders

      CELL TRANSPLANTATION
    9. Gillett, PM; Schreiber, RA; Jevon, GP; Israel, DM; Warshawski, T; Vallance, H; Clarke, LA
      Mucopolysaccharidosis type VII (Sly syndrome) presenting as neonatal cholestasis with hepatosplenomegaly

      JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
    10. Kosuga, M; Takahashi, S; Sasaki, K; Li, XK; Fujino, M; Hamada, H; Suzuki, S; Yamada, M; Matsuo, N; Okuyama, T
      Adenovirus-mediated gene therapy for mucopolysaccharidosis VII: Involvement of cross-correction in wide-spread distribution of the gene products and long-term effects of CTLA-4lg coexpression

      MOLECULAR THERAPY
    11. Bosch, A; Perret, E; Desmaris, N; Heard, JM
      Long-term and significant correction of brain lesions in adult mucopolysaccharidosis type VII mice using recombinant AAV vectors

      MOLECULAR THERAPY
    12. Sferra, TJ; Qu, G; McNeely, D; Rennard, R; Clark, KR; Lo, WD; Johnson, PR
      Recombinant adeno-associated virus-mediated correction of lysosomal storage within the central nervous system of the adult mucopolysaccharidosis typeVII mouse

      HUMAN GENE THERAPY
    13. Ross, CJD; Bastedo, L; Maier, SA; Sands, MS; Chang, PL
      Treatment of a lysosomal storage disease, mucopolysaccharidosis VII, with microencapsulated recombinant cells

      HUMAN GENE THERAPY
    14. Den Hollander, NS; Kleijer, WJ; Schoonderwaldt, EM; Los, FJ; Wladimiroff, JW; Niermeijer, MF
      In-utero diagnosis of mucopolysaccharidosis type VII in a fetus with an enlarged nuchal translucency

      ULTRASOUND IN OBSTETRICS & GYNECOLOGY
    15. Montano, AM; Yamagishi, A; Tomatsu, S; Fukuda, S; Copeland, NG; Orii, KE; Isogai, K; Yamada, N; Kato, ZI; Jenkins, NA; Gilbert, DJ; Sukegawa, K; Orii, T; Kondo, N
      The mouse N-acetylgalactosamine-6-sulfate sulfatase (Galns) gene: cDNA isolation, genomic characterization, chromosomal assignment and analysis of the 5 '-flanking region

      BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
    16. Schultheiss, PC; Gardner, SA; Owens, JM; Wenger, DA; Thrall, MA
      Mucopolysaccharidosis VII in a cat

      VETERINARY PATHOLOGY
    17. Casal, ML; Wolfe, JH
      Mucopolysaccharidosis type VII in the developing mouse fetus

      PEDIATRIC RESEARCH
    18. Daly, TM; Lorenz, RG; Sands, MS
      Abnormal immune function in vivo in a murine model of lysosomal storage disease

      PEDIATRIC RESEARCH
    19. Ross, CJD; Ralph, M; Changs, PL
      Somatic gene therapy for a neurodegenerative disease using microencapsulated recombinant cells

      EXPERIMENTAL NEUROLOGY
    20. Ohlemiller, KK; Vogler, CA; Roberts, M; Galvin, N; Sands, MS
      Retinal function is improved in a murine model of a lysosomal storage disease following bone marrow transplantation

      EXPERIMENTAL EYE RESEARCH
    21. Gwynn, B; Ciciotte, SL; Hunter, SJ; Washburn, LL; Smith, RS; Andersen, SG; Swank, RT; Dell'Angelica, EC; Bonifacino, JS; Eicher, EM; Peters, LL
      Defects in the cappuccino (cno) gene on mouse chromosome 5 and human 4p cause Hermansky-Pudlak syndrome by an AP-3-independent mechanism

      BLOOD
    22. Ohashi, T; Yokoo, T; Iizuka, S; Kobayashi, H; Sly, WS; Eto, Y
      Reduction of lysosomal storage in murine mucopolysaccharidosis type VII bytransplantation of normal and genetically modified macrophages

      BLOOD
    23. Elliger, SS; Elliger, CA; Aguilar, CP; Raju, NR; Watson, GL
      Elimination of lysosomal storage in brains of MPS VII mice treated by intrathecal administration of an adeno-associated virus vector

      GENE THERAPY
    24. Soper, BW; Duffy, TM; Vogler, CA; Barker, JE
      A genetically myeloablated MPS VII model detects the expansion and curative properties of as few as 100 enriched murine stem cells

      EXPERIMENTAL HEMATOLOGY
    25. Groener, JEM; de Graaf, FL; Poorthuis, BJHM; Kanhai, HHH
      Prenatal diagnosis of lysosomal storage diseases using fetal blood

      PRENATAL DIAGNOSIS
    26. Soper, BW; Pung, AW; Vogler, CA; Grubb, JH; Sly, WS; Barker, JE
      Enzyme replacement therapy improves reproductive performance in mucopolysaccharidosis type VII mice but does not prevent postnatal losses

      PEDIATRIC RESEARCH
    27. Stein, CS; Ghodsi, A; Derksen, T; Davidson, BL
      Systemic and central nervous system correction of lysosomal storage in mucopolysaccharidosis type VII mice

      JOURNAL OF VIROLOGY
    28. LAKE BD; YOUNG EP; WINCHESTER BG
      PRENATAL-DIAGNOSIS OF LYSOSOMAL STORAGE DISEASES

      Brain pathology
    29. SUZUKI K; PROIA RL; SUZUKI K
      MOUSE MODELS OF HUMAN LYSOSOMAL DISEASES

      Brain pathology
    30. Watson, GL; Sayles, JN; Chen, C; Elliger, SS; Elliger, CA; Raju, NR; Kurtzman, GJ; Podsakoff, GM
      Treatment of lysosomal storage disease in MPS VII mice using a recombinantadeno-associated virus

      GENE THERAPY
    31. TOKIEDA K; MORIKAWA Y; NATORI M; HAYASHIDA S; MORI K; IKEDA K
      INTRAUTERINE GROWTH ACCELERATION IN THE CASE OF A SEVERE FORM OF MUCOPOLYSACCHARIDOSIS TYPE-VII

      Journal of perinatal medicine
    32. RAY J; WOLFE JH; AGUIRRE GD; HASKINS ME
      RETROVIRAL CDNA TRANSFER TO THE RPE - STABLE EXPRESSION AND MODIFICATION OF METABOLISM

      Investigative ophthalmology & visual science
    33. VOGLER C; SANDS MS; GALVIN N; LEVY B; THORPE C; BARKER J; SLY WS
      MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII - THE IMPACT OF THERAPIES ON THE CLINICAL COURSE AND PATHOLOGY IN A MURINE MODEL OF LYSOSOMAL STORAGEDISEASE

      Journal of inherited metabolic disease
    34. FISCHER A; CARMICHAEL KP; MUNNELL JF; JHABVALA P; THOMPSON JN; MATALON R; JEZYK PF; WANG P; GIGER U
      SULFAMIDASE DEFICIENCY IN A FAMILY OF DACHSHUNDS - A CANINE MODEL OF MUCOPOLYSACCHARIDOSIS IIIA (SANFILIPPO-A)

      Pediatric research
    35. Casal, ML; Wolfe, JH
      Variant clinical course of mucopolysaccharidosis type VII in two groups ofmice carrying the same mutation

      LABORATORY INVESTIGATION
    36. RAY J; HASKINS ME; RAY K
      MOLECULAR DIAGNOSTIC-TESTS FOR ASCERTAINMENT OF GENOTYPE AT THE MUCOPOLYSACCHARIDOSIS TYPE-VII LOCUS IN DOGS

      American journal of veterinary research
    37. SNYDER EY; SENUT MC
      THE USE OF NONNEURONAL CELLS FOR GENE DELIVERY

      Neurobiology of disease
    38. CASAL ML; WOLFE JH
      AMPHOTROPIC AND ECOTROPIC RETROVIRAL VECTOR VIRUSES TRANSDUCE MIDGESTATIONAL MURINE FETAL LIVER-CELLS IN A DUAL-CHAMBERED COCULTIVATION SYSTEM

      Gene therapy
    39. SANDS MS; WOLFE JH; BIRKENMEIER EH; BARKER JE; VOGLER C; SLY WS; OKUYAMA T; FREEMAN B; NICHOLES A; MUZYCZKA N; CHANG PL; AXELROD HR
      GENE-THERAPY FOR MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII

      Neuromuscular disorders
    40. CHANG PL
      NONAUTOLOGOUS GENE-THERAPY WITH IMPLANTABLE DEVICES

      IEEE engineering in medicine and biology magazine
    41. KATTNER E; SCHAFER A; HARZER K
      HYDROPS-FETALIS - MANIFESTATION IN LYSOSOMAL STORAGE DISEASES INCLUDING FARBER-DISEASE

      European journal of pediatrics
    42. HEFFNER LJ; ROBERTS DJ; NANOWITZ M; BRAMSON RT; INSOFT RM; COLVIN RB; OGINO M
      A PREMATURE NEWBORN-INFANT WITH CONGENITAL ASCITES - GALACTOSIALIDOSIS

      The New England journal of medicine
    43. MOLYNEUX AJ; BLAIR E; COLEMAN N; DAISH P
      MUCOPOLYSACCHARIDOSIS TYPE-VII ASSOCIATED WITH HYDROPS-FETALIS - HISTOPATHOLOGICAL AND ULTRASTRUCTURAL FEATURES WITH GENETIC-IMPLICATIONS

      Journal of Clinical Pathology
    44. SANDS MS; VOGLER C; TORREY A; LEVY B; GWYNN B; GRUBB J; SLY WS; BIRKENMEIER EH
      MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII - LONG-TERM THERAPEUTIC EFFECTSOF ENZYME REPLACEMENT AND ENZYME REPLACEMENT FOLLOWED BY BONE-MARROW TRANSPLANTATION

      The Journal of clinical investigation
    45. WOLFE JH; MARTIN CE; DESHMANE SL; REILLY JJ; KESARI S; FRASER NW
      INCREASED SUSCEPTIBILITY TO THE PATHOGENIC EFFECTS OF WILD-TYPE AND RECOMBINANT HERPESVIRUSES IN MPS-VII MICE COMPARED TO NORMAL SIBLINGS

      Journal of neurovirology
    46. SNYDER EY; WOLFE JH
      CENTRAL-NERVOUS-SYSTEM CELL TRANSPLANTATION - A NOVEL THERAPY FOR STORAGE DISEASES

      Current opinion in neurology
    47. NAFFAKH N; PINSET C; MONTARRAS D; LI ZL; PAULIN D; DANOS O; HEARD JM
      LONG-TERM SECRETION OF THERAPEUTIC PROTEINS FROM GENETICALLY-MODIFIEDSKELETAL-MUSCLES

      Human gene therapy
    48. CHANG PL
      MICROENCAPSULATION - AN ALTERNATIVE APPROACH TO GENE-THERAPY

      Transfusion science
    49. LEVY B; GALVIN N; VOGLER C; BIRKENMEIER EH; SLY WS
      NEUROPATHOLOGY OF MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII

      Acta Neuropathologica
    50. ABREU S; HAYDEN J; BERTHOLD P; SHAPIRO IM; DECKER S; PATTERSON D; HASKINS M
      GROWTH-PLATE PATHOLOGY IN FELINE MUCOPOLYSACCHARIDOSIS-VI

      Calcified tissue international
    51. STEINER RD
      HYDROPS-FETALIS - ROLE OF THE GENETICIST

      Seminars in perinatology
    52. SNYDER EY; TAYLOR RM; WOLFE JH
      NEURAL PROGENITOR-CELL ENGRAFTMENT CORRECTS LYSOSOMAL STORAGE THROUGHOUT THE MPS-VII MOUSE-BRAIN

      Nature
    53. LI TS; DAVIDSON BL
      PHENOTYPE CORRECTION IN RETINAL-PIGMENT EPITHELIUM IN MURINE MUCOPOLYSACCHARIDOSIS-VII BY ADENOVIRUS-MEDIATED GENE-TRANSFER

      Proceedings of the National Academy of Sciences of the United Statesof America
    54. LANDAU D; ZEIGLER M; SHINWELL ES; MEISNER I; BARGAL R
      HYDROPS-FETALIS IN 4 SIBLINGS CAUSED BY GALACTOSIALIDOSIS

      Israel journal of medical sciences
    55. SALVETTI A; HEARD JM; DANOS O
      GENE-THERAPY OF LYSOSOMAL STORAGE DISORDERS

      British Medical Bulletin
    56. SANDS MS; ERWAY LC; VOGLER C; SLY WS; BIRKENMEIER EH
      SYNGENEIC BONE-MARROW TRANSPLANTATION REDUCES THE HEARING-LOSS ASSOCIATED WITH MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII

      Blood
    57. ROESSLER BJ; DAVIDSON BL
      DIRECT PLASMID-MEDIATED TRANSFECTION OF ADULT MURINE BRAIN-CELLS IN-VIVO USING CATIONIC LIPOSOMES

      Neuroscience letters
    58. GITZELMANN R; BOSSHARD NU; SUPERTIFURGA A; SPYCHER MA; BRINER J; WIESMANN U; LUTZ H; LITSCHI B
      FELINE MUCOPOLYSACCHARIDOSIS-VII DUE TO BETA-GLUCURONIDASE DEFICIENCY

      Veterinary pathology
    59. SHERIDAN O; WORTMAN J; HARVEY C; HAYDEN J; HASKINS M
      CRANIOFACIAL ABNORMALITIES IN ANIMAL-MODELS OF MUCOPOLYSACCHARIDOSE-I, MUCOPOLYSACCHARIDOSE-VI, AND MUCOPOLYSACCHARIDOSE-VII

      Journal of craniofacial genetics and developmental biology
    60. FENSOM AH; BENSON PF
      RECENT ADVANCES IN THE PRENATAL-DIAGNOSIS OF THE MUCOPOLYSACCHARIDOSES

      Prenatal diagnosis
    61. NORTON ME
      NONIMMUNE HYDROPS-FETALIS

      Seminars in perinatology
    62. NAFFAKH N; BOHL D; SALVETTI A; MOULLIER P; DANOS O; HEARD JM
      GENE-THERAPY FOR LYSOSOMAL DISORDERS

      Nouvelle revue francaise d'hematologie
    63. BASTEDO L; SANDS MS; LAMBERT DT; PISA MA; BIRKENMEIER E; CHANG PL
      BEHAVIORAL CONSEQUENCES OF BONE-MARROW TRANSPLANTATION IN THE TREATMENT OF MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII

      The Journal of clinical investigation
    64. SANDS MS; VOGLER C; KYLE JW; GRUBB JH; LEVY B; GALVIN N; SLY WS; BIRKENMEIER EH
      ENZYME REPLACEMENT THERAPY FOR MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII

      The Journal of clinical investigation
    65. SLY WS
      GENE-THERAPY ON THE SLY

      Nature genetics
    66. MOULLIER P; BOHL D; HEARD JM; DANOS O
      CORRECTION OF LYSOSOMAL STORAGE IN THE LIVER AND SPLEEN OF MPS-VII MICE BY IMPLANTATION OF GENETICALLY-MODIFIED SKIN FIBROBLASTS

      Nature genetics
    67. WU BM; SLY WS
      MUTATIONAL STUDIES IN A PATIENT WITH THE HYDROPS-FETALIS FORM OF MUCOPOLYSACCHARIDOSIS TYPE-VII

      Human mutation
    68. CHANG PL; LAMBERT DT; PISA MA
      BEHAVIORAL ABNORMALITIES IN A MURINE MODEL OF A HUMAN LYSOSOMAL STORAGE DISEASE

      NeuroReport
    69. BARKER JE; WOLFE JH; ROWE LB; BIRKENMEIER EH
      ADVANTAGES OF GRADIENT VS 5-FLUOROURACIL ENRICHMENT OF STEM-CELLS FORRETROVIRAL-MEDIATED GENE-TRANSFER

      Experimental hematology
    70. MOULLIER P; MARECHAL V; DANOS O; HEARD JM
      CONTINUOUS SYSTEMIC SECRETION OF A LYSOSOMAL-ENZYME BY GENETICALLY-MODIFIED MOUSE SKIN FIBROBLASTS

      Transplantation
    71. SANDS MS; BARKER JE; VOGLER C; LEVY B; GWYNN B; GALVIN N; SLY WS; BIRKENMEIER E
      TREATMENT OF MURINE MUCOPOLYSACCHARIDOSIS TYPE-VII BY SYNGENEIC BONE-MARROW TRANSPLANTATION IN NEONATES

      Laboratory investigation
    72. LAZARUS HS; SLY WS; KYLE JW; HAGEMAN GS
      PHOTORECEPTOR DEGENERATION AND ALTERED DISTRIBUTION OF INTERPHOTORECEPTOR MATRIX PROTEOGLYCANS IN THE MUCOPOLYSACCHARIDOSIS-VII MOUSE

      Experimental Eye Research
    73. MARECHAL V; NAFFAKH N; DANOS O; HEARD JM
      DISAPPEARANCE OF LYSOSOMAL STORAGE IN SPLEEN AND LIVER OF MUCOPOLYSACCHARIDOSIS-VII MICE AFTER TRANSPLANTATION OF GENETICALLY-MODIFIED BONE-MARROW CELLS

      Blood
    74. MOULLIER P; MARECHAL V; FERRY N; NAFFAKH N; HEARD JM; DANOS O
      GENE-TRANSFER TO SOMATIC TISSUES USING RETROVIRAL VECTORS

      Nouvelle revue francaise d'hematologie


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Documento generato il 08/08/20 alle ore 09:07:30