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Items: 1 to 20 of 355

1.

Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism.

Sango K, Yamanaka S, Hoffmann A, Okuda Y, Grinberg A, Westphal H, McDonald MP, Crawley JN, Sandhoff K, Suzuki K, Proia RL.

Nat Genet. 1995 Oct;11(2):170-6.

PMID:
7550345
2.

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
3.

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.

PMID:
9302266
4.

Characterization of the testis and epididymis in mouse models of human Tay Sachs and Sandhoff diseases and partial determination of accumulated gangliosides.

Trasler J, Saberi F, Somani IH, Adamali HI, Huang JQ, Fortunato SR, Ritter G, Gu M, Aebersold R, Gravel RA, Hermo L.

Endocrinology. 1998 Jul;139(7):3280-8.

PMID:
9645704
5.
6.

Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis.

Sango K, McDonald MP, Crawley JN, Mack ML, Tifft CJ, Skop E, Starr CM, Hoffmann A, Sandhoff K, Suzuki K, Proia RL.

Nat Genet. 1996 Nov;14(3):348-52.

PMID:
8896570
7.

Characterization of inducible models of Tay-Sachs and related disease.

Sargeant TJ, Drage DJ, Wang S, Apostolakis AA, Cox TM, Cachón-González MB.

PLoS Genet. 2012 Sep;8(9):e1002943. doi: 10.1371/journal.pgen.1002943. Epub 2012 Sep 20.

8.

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
9.
10.

[Molecular pathogenesis and therapeutic approach of GM2 gangliosidosis].

Tsuji D.

Yakugaku Zasshi. 2013;133(2):269-74. Review. Japanese.

11.

[Recent advances in molecular genetics of GM2 gangliosidosis].

Wakamatsu N.

Nihon Rinsho. 1995 Dec;53(12):2988-93. Review. Japanese.

PMID:
8577047
12.

[Beta-N-acetyl-hexosaminidase--the enzyme of Tay-Sachs and Sandhoff diseases].

Zwierz K, Juszkiewicz J, Arciuch L, Gindzieński A.

Postepy Biochem. 1992;38(3):127-32. Review. Polish. No abstract available.

PMID:
1461844
13.

An inducible mouse model of late onset Tay-Sachs disease.

Jeyakumar M, Smith D, Eliott-Smith E, Cortina-Borja M, Reinkensmeier G, Butters TD, Lemm T, Sandhoff K, Perry VH, Dwek RA, Platt FM.

Neurobiol Dis. 2002 Aug;10(3):201-10.

PMID:
12270683
14.

Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff disease.

Buccoliero R, Bodennec J, Van Echten-Deckert G, Sandhoff K, Futerman AH.

J Neurochem. 2004 Jul;90(1):80-8.

15.

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.

16.

Structure and expression of the mouse beta-hexosaminidase genes, Hexa and Hexb.

Yamanaka S, Johnson ON, Norflus F, Boles DJ, Proia RL.

Genomics. 1994 Jun;21(3):588-96.

PMID:
7959736
17.

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.

18.

Promoters for the human beta-hexosaminidase genes, HEXA and HEXB.

Norflus F, Yamanaka S, Proia RL.

DNA Cell Biol. 1996 Feb;15(2):89-97.

PMID:
8634145
19.

The GM2 gangliosidoses databases: allelic variation at the HEXA, HEXB, and GM2A gene loci.

Cordeiro P, Hechtman P, Kaplan F.

Genet Med. 2000 Nov-Dec;2(6):319-27. Review.

PMID:
11339652
20.

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.

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