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Journal Abstract Search

177 related items for PubMed ID: 12374215

  • 1. Restoration of the GM2 ganglioside metabolism in bone marrow-derived stromal cells from Tay-Sachs disease animal model.
    Martino S, Cavalieri C, Emiliani C, Dolcetta D, Cusella De Angelis MG, Chigorno V, Severini GM, Sandhoff K, Bordignon C, Sonnino S, Orlacchio A.
    Neurochem Res; 2002 Aug; 27(7-8):793-800. PubMed ID: 12374215
    [Abstract] [Full Text] [Related]

  • 2. Absence of metabolic cross-correction in Tay-Sachs cells: implications for gene therapy.
    Martino S, Emiliani C, Tancini B, Severini GM, Chigorno V, Bordignon C, Sonnino S, Orlacchio A.
    J Biol Chem; 2002 Jun 07; 277(23):20177-84. PubMed ID: 11923278
    [Abstract] [Full Text] [Related]

  • 3. In cellulo examination of a beta-alpha hybrid construct of beta-hexosaminidase A subunits, reported to interact with the GM2 activator protein and hydrolyze GM2 ganglioside.
    Sinici I, Yonekawa S, Tkachyova I, Gray SJ, Samulski RJ, Wakarchuk W, Mark BL, Mahuran DJ.
    PLoS One; 2013 Jun 07; 8(3):e57908. PubMed ID: 23483939
    [Abstract] [Full Text] [Related]

  • 4. GM2 ganglioside accumulation causes neuroinflammation and behavioral alterations in a mouse model of early onset Tay-Sachs disease.
    Demir SA, Timur ZK, Ateş N, Martínez LA, Seyrantepe V.
    J Neuroinflammation; 2020 Sep 20; 17(1):277. PubMed ID: 32951593
    [Abstract] [Full Text] [Related]

  • 5. Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease.
    Kodama T, Togawa T, Tsukimura T, Kawashima I, Matsuoka K, Kitakaze K, Tsuji D, Itoh K, Ishida Y, Suzuki M, Suzuki T, Sakuraba H.
    PLoS One; 2011 Sep 20; 6(12):e29074. PubMed ID: 22205997
    [Abstract] [Full Text] [Related]

  • 6. Autophagic flux is impaired in the brain tissue of Tay-Sachs disease mouse model.
    Sengul T, Can M, Ateş N, Seyrantepe V.
    PLoS One; 2023 Sep 20; 18(3):e0280650. PubMed ID: 36928510
    [Abstract] [Full Text] [Related]

  • 7. Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin.
    Platt FM, Neises GR, Reinkensmeier G, Townsend MJ, Perry VH, Proia RL, Winchester B, Dwek RA, Butters TD.
    Science; 1997 Apr 18; 276(5311):428-31. PubMed ID: 9103204
    [Abstract] [Full Text] [Related]

  • 8. Tay-Sachs disease brain cells in culture: mobilization of stored GM2 after concanavalin A-mediated uptake of hexosaminidase A.
    Hoffman LM, Brooks SE, Amsterdam D, Oropello J, Schneck L.
    J Neurosci Res; 1980 Apr 18; 5(5):413-7. PubMed ID: 7441795
    [Abstract] [Full Text] [Related]

  • 9. Effective gene therapy in an authentic model of Tay-Sachs-related diseases.
    Cachón-González MB, Wang SZ, Lynch A, Ziegler R, Cheng SH, Cox TM.
    Proc Natl Acad Sci U S A; 2006 Jul 05; 103(27):10373-10378. PubMed ID: 16801539
    [Abstract] [Full Text] [Related]

  • 10. Therapeutic potential of intracerebroventricular replacement of modified human β-hexosaminidase B for GM2 gangliosidosis.
    Matsuoka K, Tamura T, Tsuji D, Dohzono Y, Kitakaze K, Ohno K, Saito S, Sakuraba H, Itoh K.
    Mol Ther; 2011 Jun 05; 19(6):1017-24. PubMed ID: 21487393
    [Abstract] [Full Text] [Related]

  • 11. Evidence for direct binding of intracellularly distributed ganglioside GM2 to isolated vimentin intermediate filaments in normal and Tay-Sachs disease human fibroblasts.
    Kotani M, Hosoya H, Kubo H, Itoh K, Sakuraba H, Kusubata M, Inagaki M, Yazaki S, Suzuki Y, Tai T.
    Cell Struct Funct; 1994 Apr 05; 19(2):81-7. PubMed ID: 7923401
    [Abstract] [Full Text] [Related]

  • 12. Progranulin associates with hexosaminidase A and ameliorates GM2 ganglioside accumulation and lysosomal storage in Tay-Sachs disease.
    Chen Y, Jian J, Hettinghouse A, Zhao X, Setchell KDR, Sun Y, Liu CJ.
    J Mol Med (Berl); 2018 Dec 05; 96(12):1359-1373. PubMed ID: 30341570
    [Abstract] [Full Text] [Related]

  • 13. Sialidase-mediated depletion of GM2 ganglioside in Tay-Sachs neuroglia cells.
    Igdoura SA, Mertineit C, Trasler JM, Gravel RA.
    Hum Mol Genet; 1999 Jun 05; 8(6):1111-6. PubMed ID: 10332044
    [Abstract] [Full Text] [Related]

  • 14. Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases.
    Huang JQ, Trasler JM, Igdoura S, Michaud J, Hanal N, Gravel RA.
    Hum Mol Genet; 1997 Oct 05; 6(11):1879-85. PubMed ID: 9302266
    [Abstract] [Full Text] [Related]

  • 15. Novel Vector Design and Hexosaminidase Variant Enabling Self-Complementary Adeno-Associated Virus for the Treatment of Tay-Sachs Disease.
    Karumuthil-Melethil S, Nagabhushan Kalburgi S, Thompson P, Tropak M, Kaytor MD, Keimel JG, Mark BL, Mahuran D, Walia JS, Gray SJ.
    Hum Gene Ther; 2016 Jul 05; 27(7):509-21. PubMed ID: 27197548
    [Abstract] [Full Text] [Related]

  • 16. Biology and potential strategies for the treatment of GM2 gangliosidoses.
    Chavany C, Jendoubi M.
    Mol Med Today; 1998 Apr 05; 4(4):158-65. PubMed ID: 9572057
    [Abstract] [Full Text] [Related]

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  • 18. Murine Sialidase Neu3 facilitates GM2 degradation and bypass in mouse model of Tay-Sachs disease.
    Seyrantepe V, Demir SA, Timur ZK, Von Gerichten J, Marsching C, Erdemli E, Oztas E, Takahashi K, Yamaguchi K, Ates N, Dönmez Demir B, Dalkara T, Erich K, Hopf C, Sandhoff R, Miyagi T.
    Exp Neurol; 2018 Jan 05; 299(Pt A):26-41. PubMed ID: 28974375
    [Abstract] [Full Text] [Related]

  • 19. Lithium treatment rescues dysfunctional autophagy in the cell models of Tay-Sachs disease.
    Basirli H, Can M, Sengul T, Seyrantepe V.
    Mol Genet Metab; 2024 Mar 05; 141(3):108140. PubMed ID: 38262289
    [Abstract] [Full Text] [Related]

  • 20. Composition of gangliosides and neutral glycosphingolipids of brain in classical Tay-Sachs and Sandhoff disease: more lyso-GM2 in Sandhoff disease?
    Rosengren B, Månsson JE, Svennerholm L.
    J Neurochem; 1987 Sep 05; 49(3):834-40. PubMed ID: 3612128
    [Abstract] [Full Text] [Related]

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