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La ricerca find articoli where authors phrase all words ' PROIA RL' sort by level,fasc_key/DESCEND, pagina_ini_num/ASCEND ha restituito 45 riferimenti
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    1. Proia, RL
      Cloning the beta-hexosaminidase genes

      TAY-SACHS DISEASE
    2. Proia, RL
      Targeting the hexosaminidase genes: Mouse models of the G(M2) gangliosidoses

      TAY-SACHS DISEASE
    3. Kawai, H; Allende, ML; Wada, R; Kono, M; Sango, K; Deng, CX; Miyakawa, T; Crawley, JN; Werth, N; Bierfreund, U; Sandhoff, K; Proia, RL
      Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures

      JOURNAL OF BIOLOGICAL CHEMISTRY
    4. Jeyakumar, M; Norflus, F; Tifft, CJ; Cortina-Borja, M; Butters, TD; Proia, RL; Perry, VH; Dwek, RA; Platt, FM
      Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation

      BLOOD
    5. Liu, YJ; Wu, YP; Wada, R; Neufeld, EB; Mullin, KA; Howard, AC; Pentchev, PG; Vanier, MT; Suzuki, K; Proia, RL
      Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse

      HUMAN MOLECULAR GENETICS
    6. Tifft, CJ; Proia, RL
      Stemming the tide: glycosphingolipid synthesis inhibitors as therapy for storage diseases

      GLYCOBIOLOGY
    7. Potratz, A; Huttler, S; Bierfreund, U; Proia, RL; Suzuki, K; Sandhoff, K
      Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency

      BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
    8. Wada, R; Tifft, CJ; Proia, RL
      Microglial activation precedes acute neurodegeneration in Sandhoff diseaseand is suppressed by bone marrow transplantation

      PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
    9. Liu, YJ; Wada, R; Yamashita, T; Mi, YD; Deng, CX; Hobson, JP; Rosenfeldt, HM; Nava, VE; Chae, SS; Lee, MJ; Liu, CH; Hla, T; Spiegel, S; Proia, RL
      Edg-1, the G protein-coupled receptor for sphingosine-1-phosphate, is essential for vascular maturation

      JOURNAL OF CLINICAL INVESTIGATION
    10. Oya, Y; Proia, RL; Norflus, F; Tifft, CJ; Langaman, C; Suzuki, K
      Distribution of enzyme-bearing cells in GM(2) gangliosidosis mice: regionally specific pattern of cellular infiltration following bone marrow transplantation

      ACTA NEUROPATHOLOGICA
    11. Yamashita, T; Wada, R; Sasaki, T; Deng, CX; Bierfreund, U; Sandhoff, K; Proia, RL
      A vital role for glycosphingolipid synthesis during development and differentiation

      PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
    12. Sheikh, KA; Sun, J; Liu, YJ; Kawai, H; Crawford, TO; Proia, RL; Griffin, JW; Schnaar, RL
      Mice lacking complex gangliosides develop Wallerian degeneration and myelination defects

      PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
    13. Jeyakumar, M; Butters, TD; Cortina-Borja, M; Hunnam, V; Proia, RL; Perry, VH; Dwek, RA; Platt, FM
      Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin

      PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
    14. Liu, YJ; Wada, R; Kawai, H; Sango, K; Deng, CX; Tai, T; McDonald, MP; Araujo, K; Crawley, JN; Bierfreund, U; Sandhoff, K; Suzuki, K; Proia, RL
      A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder

      JOURNAL OF CLINICAL INVESTIGATION
    15. Doering, T; Proia, RL; Sandhoff, K
      Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher mice

      FEBS LETTERS
    16. SUZUKI K; PROIA RL; SUZUKI K
      MOUSE MODELS OF HUMAN LYSOSOMAL DISEASES

      Brain pathology
    17. LIU YJ; SUZUKI K; REED JD; GRINBERG A; WESTPHAL H; HOFFMANN A; DORING T; SANDHOFF K; PROIA RL
      MICE WITH TYPE-2 AND TYPE-3 GAUCHER-DISEASE POINT MUTATIONS GENERATEDBY A SINGLE INSERTION MUTAGENESIS PROCEDURE (SIMP)

      Proceedings of the National Academy of Sciences of the United Statesof America
    18. NORFLUS F; TIFFT CJ; MCDONALD MP; GOLDSTEIN G; CRAWLEY JN; HOFFMANN A; SANDHOFF K; SUZUKI K; PROIA RL
      BONE-MARROW TRANSPLANTATION PROLONGS LIFE-SPAN AND AMELIORATES NEUROLOGIC MANIFESTATIONS IN SANDHOFF DISEASE MICE

      The Journal of clinical investigation
    19. KAWAI H; SANGO K; MULLIN KA; PROIA RL
      EMBRYONIC STEM-CELLS WITH A DISRUPTED GD3 SYNTHASE GENE UNDERGO NEURONAL DIFFERENTIATION IN THE ABSENCE OF B-SERIES GANGLIOSIDES

      The Journal of biological chemistry
    20. DRUCKER L; GOLAN A; BOLES DJ; ELBEDOUR K; PROIA RL; NAVON R
      NOVEL HEXA MUTATION IN A BEDOUIN TAY-SACHS PATIENT ASSOCIATED WITH EXON SKIPPING AND REDUCED TRANSCRIPT LEVEL

      Human mutation
    21. NARKIS G; ADAM A; JABER L; PENNYBACKER M; PROIA RL; NAVON R
      MOLECULAR-BASIS OF HEAT-LABILE HEXOSAMINIDASE-B AMONG JEWS AND ARABS

      Human mutation
    22. TIFFT CJ; PROIA RL
      THE BETA-HEXOSAMINIDASE DEFICIENCY DISORDERS - DEVELOPMENT OF A CLINICAL PARADIGM IN THE MOUSE

      Annals of medicine
    23. PLATT FM; NEISES GR; REINKENSMEIER G; TOWNSEND MJ; PERRY VH; PROIA RL; WINCHESTER B; DWEK RA; BUTTERS TD
      PREVENTION OF LYSOSOMAL STORAGE IN TAY-SACHS MICE TREATED WITH N-BUTYLDEOXYNOJIRIMYCIN

      Science
    24. LIU YJ; HOFFMANN A; GRINBERG A; WESTPHAL H; MCDONALD MP; MILLER KM; CRAWLEY JN; SANDHOFF K; SUZUKI K; PROIA RL
      MOUSE MODEL OF G(M2) ACTIVATOR DEFICIENCY MANIFESTS CEREBELLAR PATHOLOGY AND MOTOR IMPAIRMENT

      Proceedings of the National Academy of Sciences of the United Statesof America
    25. SUZUKI K; SANGO K; PROIA RL; LANGAMAN C
      MICE DEFICIENT IN ALL FORMS OF LYSOSOMAL BETA-HEXOSAMINIDASE SHOW MUCOPOLYSACCHARIDOSIS-LIKE PATHOLOGY

      Journal of neuropathology and experimental neurology
    26. PENNYBACKER M; SCHUETTE CG; LIESSEM B; HEPBILDIKLER ST; KOPETKA JA; ELLIS MR; MYEROWITZ R; SANDHOFF K; PROIA RL
      EVIDENCE FOR THE INVOLVEMENT OF GLU-355 IN THE CATALYTIC ACTION OF HUMAN BETA-HEXOSAMINIDASE-B

      The Journal of biological chemistry
    27. LIU Y; GRINBERG A; WESTPHAL H; PROIA RL
      MODELING THE CLINICAL FORMS OF GAUCHER-DISEASE - INTRODUCTION OF HUMAN-DISEASE MUTATIONS BY A SINGLE INSERTION MUTAGENESIS PROCEDURE (SIMP)

      American journal of human genetics
    28. SANGO K; MCDONALD MP; CRAWLEY JN; MACK ML; TIFFT CJ; SKOP E; STARR CM; HOFFMANN A; SANDHOFF K; SUZUKI K; PROIA RL
      MICE LACKING BOTH SUBUNITS OF LYSOSOMAL BETA-HEXOSAMINIDASE DISPLAY GANGLIOSIDOSIS AND MUCOPOLYSACCHARIDOSIS

      Nature genetics
    29. NORFLUS F; YAMANAKA S; PROIA RL
      PROMOTERS FOR THE HUMAN BETA-HEXOSAMINIDASE GENES, HEXA AND HEXB

      DNA and cell biology
    30. PLATT FM; NEISES GR; PROIA RL; DWEK RA; BUTTERS TD
      NOVEL INHIBITORS OF GLYCOSPHINGOLIPID BIOSYNTHESIS

      Glycobiology
    31. SUZUKI K; HOFFMANN A; SANDHOFF K; STARR CM; SKOP E; SANGO K; PROIA RL
      MICE TOTALLY DEFICIENT IN BETA-HEXOSAMINIDASE ALPHA-SUBUNIT AND BETA-SUBUNIT SHOW AN ADDITIONAL MUCOPOLYSACCHARIDOSIS PHENOTYPE

      Journal of neuropathology and experimental neurology
    32. PENNYBACKER M; LIESSEM B; MOCZALL H; TIFFT CJ; SANDHOFF K; PROIA RL
      IDENTIFICATION OF DOMAINS IN HUMAN BETA-HEXOSAMINIDASE THAT DETERMINESUBSTRATE-SPECIFICITY

      The Journal of biological chemistry
    33. SANGO K; YAMANAKA S; HOFFMANN A; OKUDA Y; GRINBERG A; WESTPHAL H; MCDONALD MP; CRAWLEY JN; SANDHOFF K; SUZUKI K; PROIA RL
      MOUSE MODELS OF TAY-SACHS AND SANDHOFF DISEASES DIFFER IN NEUROLOGIC PHENOTYPE AND GANGLIOSIDE METABOLISM

      Nature genetics
    34. SANGO K; JOHNSON ON; KOZAK CA; PROIA RL
      BETA-1,4-N-ACETYLGALACTOSAMINYLTRANSFERASE INVOLVED IN GANGLIOSIDE SYNTHESIS - CDNA SEQUENCE, EXPRESSION, AND CHROMOSOME MAPPING OF THE MOUSE GENE

      Genomics
    35. SNYDER EY; MACKLIS ID; WOLFE JH; GINNS EI; JENDOUBI M; SIDMAN RL; TESSLER A; PROIA RL; GOTTLIEB DI; FRIEDMANN T; YANDAVA BD; FLAX JD; AURORA S; YOON CH; MOZELL RL; PAN ZH; TAYLOR RM; MCKINNEY C; LACORAZZA HD; ROSARIO CM; KOSARAS B; KITCHENS DL; BAUM L
      CNS PROGENITOR AND STEM-LIKE CELLS AS GENE DELIVERY VEHICLES AND MEDIATORS OF REPAIR

      Journal of cellular biochemistry
    36. PROIA RL; SANGO K; YAMANAKA S; OKUDA Y; GRINBERG A; WESTPHAL H; MCDONALD M; CRAWLEY JN; SUZUKI K
      MOUSE MODELS OF TAY-SACHS AND SANDHOFF DISEASES DISPLAY VASTLY DIFFERENT PHENOTYPES

      American journal of human genetics
    37. BOLES DJ; PROIA RL
      THE MOLECULAR-BASIS OF HUXA MESSENGER-RNA DEFICIENCY CAUSED BY THE MOST COMMON TAY-SACHS-DISEASE MUTATION

      American journal of human genetics
    38. TANIIKE M; YAMANAKA S; PROIA RL; LANGAMAN C; BONETURRENTINE T; SUZUKI K
      NEUROPATHOLOGY OF MICE WITH TARGETED DISRUPTION OF HEXA GENE, A MODELOF TAY-SACHS-DISEASE

      Acta Neuropathologica
    39. BOLES DJ; PROIA RL
      STABILITY AND PROCESSING OF BETA-HEXOSAMINIDASE-A MESSENGER-RNA IN THE PRESENCE OF A COMMON 4BP INSERTION

      The FASEB journal
    40. NORFLUS F; YAMANAKA S; PROIA RL
      CHARACTERIZATION OF THE PROMOTERS FOR THE BETA-HEXOSAMINIDASE GENES, HEXA AND HEXB

      The FASEB journal
    41. PENNYBACKER M; PROIA RL
      LOCALIZATION OF SUBSTRATE-SPECIFICITY DOMAINS OF BETA-HEXOSAMINIDASE USING CHIMERIC ENZYME CONSTRUCTS

      The FASEB journal
    42. YAMANAKA S; JOHNSON ON; LYU MS; KOZAK CA; PROIA RL
      THE MOUSE GENE ENCODING THE G(M2) ACTIVATOR PROTEIN (GM2A) - CDNA SEQUENCE, EXPRESSION, AND CHROMOSOME MAPPING

      Genomics
    43. YAMANAKA S; JOHNSON ON; NORFLUS F; BOLES DJ; PROIA RL
      STRUCTURE AND EXPRESSION OF THE MOUSE BETA-HEXOSAMINIDASE GENES, HEXAAND HEXB

      Genomics
    44. YAMANAKA S; JOHNSON MD; GRINBERG A; WESTPHAL H; CRAWLEY JN; TANIIKE M; SUZUKI K; PROIA RL
      TARGETED DISRUPTION OF THE HEXA GENE RESULTS IN MICE WITH BIOCHEMICALAND PATHOLOGICAL FEATURES OF TAY-SACHS-DISEASE

      Proceedings of the National Academy of Sciences of the United Statesof America
    45. BOLES DJ; PROIA RL
      PROCESSING OF HEXA MESSENGER-RNA IN THE PRESENCE OF THE 4BP INSERTIONCOMMONLY FOUND IN TAY-SACHS-DISEASE

      American journal of human genetics


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Documento generato il 18/01/21 alle ore 00:19:01