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Items: 1 to 20 of 113

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.

PMID:
12374215
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 7;277(23):20177-84. Epub 2002 Mar 28.

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;8(3):e57908. doi: 10.1371/journal.pone.0057908. Epub 2013 Mar 4.

4.

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;6(12):e29074. doi: 10.1371/journal.pone.0029074. Epub 2011 Dec 20.

5.

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 5;103(27):10373-8. Epub 2006 Jun 26.

6.

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.

7.

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;5(5):413-7.

PMID:
7441795
8.

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;19(6):1017-24. doi: 10.1038/mt.2011.27. Epub 2011 Apr 12.

9.

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;19(2):81-7.

10.

Sialidase-mediated depletion of GM2 ganglioside in Tay-Sachs neuroglia cells.

Igdoura SA, Mertineit C, Trasler JM, Gravel RA.

Hum Mol Genet. 1999 Jun;8(6):1111-6.

11.

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;6(11):1879-85.

12.

Biology and potential strategies for the treatment of GM2 gangliosidoses.

Chavany C, Jendoubi M.

Mol Med Today. 1998 Apr;4(4):158-65. Review.

PMID:
9572057
13.

Late onset Tay-Sachs disease in mice with targeted disruption of the Hexa gene: behavioral changes and pathology of the central nervous system.

Miklyaeva EI, Dong W, Bureau A, Fattahie R, Xu Y, Su M, Fick GH, Huang JQ, Igdoura S, Hanai N, Gravel RA.

Brain Res. 2004 Mar 19;1001(1-2):37-50.

PMID:
14972652
14.

Diagnosis of Tay-Sachs disease using [3H]N-acetylneuraminic acid labelled GM2 ganglioside as substrate.

Poulos A, Holding J, Carey WF.

Clin Chim Acta. 1982 Apr 23;120(3):331-40.

PMID:
6210469
15.

Neuropathology of mice with targeted disruption of Hexa gene, a model of Tay-Sachs disease.

Taniike M, Yamanaka S, Proia RL, Langaman C, Bone-Turrentine T, Suzuki K.

Acta Neuropathol. 1995;89(4):296-304.

PMID:
7610760
16.

Restoration of hexosaminidase A activity in human Tay-Sachs fibroblasts via adenoviral vector-mediated gene transfer.

Akli S, Guidotti JE, Vigne E, Perricaudet M, Sandhoff K, Kahn A, Poenaru L.

Gene Ther. 1996 Sep;3(9):769-74.

PMID:
8875224
17.

Naturally occurring GM2 gangliosidosis in two Muntjak deer with pathological and biochemical features of human classical Tay-Sachs disease (type B GM2 gangliosidosis).

Fox J, Li YT, Dawson G, Alleman A, Johnsrude J, Schumacher J, Homer B.

Acta Neuropathol. 1999 Jan;97(1):57-62.

PMID:
9930895
18.
20.

Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment.

Liu Y, Hoffmann A, Grinberg A, Westphal H, McDonald MP, Miller KM, Crawley JN, Sandhoff K, Suzuki K, Proia RL.

Proc Natl Acad Sci U S A. 1997 Jul 22;94(15):8138-43.

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