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Results: 1 to 20 of 148

1.

Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation.

Jeyakumar M, Norflus F, Tifft CJ, Cortina-Borja M, Butters TD, Proia RL, Perry VH, Dwek RA, Platt FM.

Blood. 2001 Jan 1;97(1):327-9.

PMID:
11133779
[PubMed - indexed for MEDLINE]
Free Article
2.

Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.

Jeyakumar M, Butters TD, Cortina-Borja M, Hunnam V, Proia RL, Perry VH, Dwek RA, Platt FM.

Proc Natl Acad Sci U S A. 1999 May 25;96(11):6388-93.

PMID:
10339597
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Restricted ketogenic diet enhances the therapeutic action of N-butyldeoxynojirimycin towards brain GM2 accumulation in adult Sandhoff disease mice.

Denny CA, Heinecke KA, Kim YP, Baek RC, Loh KS, Butters TD, Bronson RT, Platt FM, Seyfried TN.

J Neurochem. 2010 Jun;113(6):1525-35. doi: 10.1111/j.1471-4159.2010.06733.x. Epub 2010 Apr 3.

PMID:
20374428
[PubMed - indexed for MEDLINE]
4.

Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease.

Andersson U, Smith D, Jeyakumar M, Butters TD, Borja MC, Dwek RA, Platt FM.

Neurobiol Dis. 2004 Aug;16(3):506-15.

PMID:
15262262
[PubMed - indexed for MEDLINE]
5.

N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice.

Baek RC, Kasperzyk JL, Platt FM, Seyfried TN.

Neurochem Int. 2008 May;52(6):1125-33. doi: 10.1016/j.neuint.2007.12.001. Epub 2007 Dec 8.

PMID:
18207611
[PubMed - indexed for MEDLINE]
6.

Therapeutic effects of stem cells and substrate reduction in juvenile Sandhoff mice.

Arthur JR, Lee JP, Snyder EY, Seyfried TN.

Neurochem Res. 2012 Jun;37(6):1335-43. doi: 10.1007/s11064-012-0718-0. Epub 2012 Feb 25.

PMID:
22367451
[PubMed - indexed for MEDLINE]
7.

Substrate reduction therapy for glycosphingolipid storage disorders.

Lachmann RH, Platt FM.

Expert Opin Investig Drugs. 2001 Mar;10(3):455-66. Review.

PMID:
11227045
[PubMed - indexed for MEDLINE]
8.

Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin.

Pelled D, Lloyd-Evans E, Riebeling C, Jeyakumar M, Platt FM, Futerman AH.

J Biol Chem. 2003 Aug 8;278(32):29496-501. Epub 2003 May 19.

PMID:
12756243
[PubMed - indexed for MEDLINE]
Free Article
9.

NSAIDs increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin.

Jeyakumar M, Smith DA, Williams IM, Borja MC, Neville DC, Butters TD, Dwek RA, Platt FM.

Ann Neurol. 2004 Nov;56(5):642-9. Erratum in: Ann Neurol. 2005 Jan;57(1):156.

PMID:
15505823
[PubMed - indexed for MEDLINE]
10.

Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.

Norflus F, Tifft CJ, McDonald MP, Goldstein G, Crawley JN, Hoffmann A, Sandhoff K, Suzuki K, Proia RL.

J Clin Invest. 1998 May 1;101(9):1881-8.

PMID:
9576752
[PubMed - indexed for MEDLINE]
Free PMC Article
11.

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.

PMID:
9103204
[PubMed - indexed for MEDLINE]
Free Article
12.

Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.

Jeyakumar M, Butters TD, Dwek RA, Platt FM.

Neuropathol Appl Neurobiol. 2002 Oct;28(5):343-57. Review.

PMID:
12366816
[PubMed - indexed for MEDLINE]
13.

Plasmid-based gene transfer ameliorates visceral storage in a mouse model of Sandhoff disease.

Yamaguchi A, Katsuyama K, Suzuki K, Kosaka K, Aoki I, Yamanaka S.

J Mol Med (Berl). 2003 Mar;81(3):185-93. Epub 2003 Feb 12.

PMID:
12682727
[PubMed - indexed for MEDLINE]
14.

Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation.

Wada R, Tifft CJ, Proia RL.

Proc Natl Acad Sci U S A. 2000 Sep 26;97(20):10954-9.

PMID:
11005868
[PubMed - indexed for MEDLINE]
Free PMC Article
15.

Metabolic correction in microglia derived from Sandhoff disease model mice.

Tsuji D, Kuroki A, Ishibashi Y, Itakura T, Itoh K.

J Neurochem. 2005 Sep;94(6):1631-8. Epub 2005 Aug 10.

PMID:
16092933
[PubMed - indexed for MEDLINE]
16.

Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases.

Phaneuf D, Wakamatsu N, Huang JQ, Borowski A, Peterson AC, Fortunato SR, Ritter G, Igdoura SA, Morales CR, Benoit G, Akerman BR, Leclerc D, Hanai N, Marth JD, Trasler JM, Gravel RA.

Hum Mol Genet. 1996 Jan;5(1):1-14.

PMID:
8789434
[PubMed - indexed for MEDLINE]
Free Article
17.

Lysosomal storage of oligosaccharide and glycosphingolipid in imino sugar treated cells.

Boomkamp SD, Rountree JS, Neville DC, Dwek RA, Fleet GW, Butters TD.

Glycoconj J. 2010 Apr;27(3):297-308. doi: 10.1007/s10719-010-9278-1. Epub 2010 Feb 26.

PMID:
20186478
[PubMed - indexed for MEDLINE]
18.

Specific induction of macrophage inflammatory protein 1-alpha in glial cells of Sandhoff disease model mice associated with accumulation of N-acetylhexosaminyl glycoconjugates.

Tsuji D, Kuroki A, Ishibashi Y, Itakura T, Kuwahara J, Yamanaka S, Itoh K.

J Neurochem. 2005 Mar;92(6):1497-507.

PMID:
15748167
[PubMed - indexed for MEDLINE]
19.

Substrate reduction therapy in mouse models of the glycosphingolipidoses.

Platt FM, Jeyakumar M, Andersson U, Heare T, Dwek RA, Butters TD.

Philos Trans R Soc Lond B Biol Sci. 2003 May 29;358(1433):947-54. Review.

PMID:
12803928
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

Distribution of enzyme-bearing cells in GM2 gangliosidosis mice: regionally specific pattern of cellular infiltration following bone marrow transplantation.

Oya Y, Proia RL, Norflus F, Tifft CJ, Langaman C, Suzuki K.

Acta Neuropathol. 2000 Feb;99(2):161-8.

PMID:
10672323
[PubMed - indexed for MEDLINE]

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