398 related articles for article (PubMed ID: 28974375)
1. 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; 299(Pt A):26-41. PubMed ID: 28974375
[TBL] [Abstract][Full Text] [Related]
2. 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; 17(1):277. PubMed ID: 32951593
[TBL] [Abstract][Full Text] [Related]
3. 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; 1001(1-2):37-50. PubMed ID: 14972652
[TBL] [Abstract][Full Text] [Related]
4. 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. PubMed ID: 8789434
[TBL] [Abstract][Full Text] [Related]
5. Analysis of Brain Lipids in the Early-Onset Tay-Sachs Disease Mouse Model With the Combined Deficiency of β-Hexosaminidase A and Neuraminidase 3.
Can M; Sengül T; Demir SA; İnci OK; Basırlı H; Seyrantepe V
Front Mol Biosci; 2022; 9():892248. PubMed ID: 36003081
[No Abstract] [Full Text] [Related]
6. [Molecular pathogenesis and therapeutic approach of GM2 gangliosidosis].
Tsuji D
Yakugaku Zasshi; 2013; 133(2):269-74. PubMed ID: 23370522
[TBL] [Abstract][Full Text] [Related]
7. 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; 18(3):e0280650. PubMed ID: 36928510
[TBL] [Abstract][Full Text] [Related]
8. A pathogenic HEXA missense variant in wild boars with Tay-Sachs disease.
Bertani V; Prioni S; Di Lecce R; Gazza F; Ragionieri L; Merialdi G; Bonilauri P; Jagannathan V; Grassi S; Cabitta L; Paoli A; Morrone A; Sonnino S; Drögemüller C; Cantoni AM
Mol Genet Metab; 2021 Jul; 133(3):297-306. PubMed ID: 34119419
[TBL] [Abstract][Full Text] [Related]
9. 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; 141(3):108140. PubMed ID: 38262289
[TBL] [Abstract][Full Text] [Related]
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. PubMed ID: 10332044
[TBL] [Abstract][Full Text] [Related]
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. PubMed ID: 9302266
[TBL] [Abstract][Full Text] [Related]
12. 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; 94(15):8138-43. PubMed ID: 9223328
[TBL] [Abstract][Full Text] [Related]
13. Disruption of murine Hexa gene leads to enzymatic deficiency and to neuronal lysosomal storage, similar to that observed in Tay-Sachs disease.
Cohen-Tannoudji M; Marchand P; Akli S; Sheardown SA; Puech JP; Kress C; Gressens P; Nassogne MC; Beccari T; Muggleton-Harris AL
Mamm Genome; 1995 Dec; 6(12):844-9. PubMed ID: 8747922
[TBL] [Abstract][Full Text] [Related]
14. GM2 gangliosidosis associated with a HEXA missense mutation in Japanese Chin dogs: a potential model for Tay Sachs disease.
Sanders DN; Zeng R; Wenger DA; Johnson GS; Johnson GC; Decker JE; Katz ML; Platt SR; O'Brien DP
Mol Genet Metab; 2013 Jan; 108(1):70-5. PubMed ID: 23266199
[TBL] [Abstract][Full Text] [Related]
15. 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; 96(12):1359-1373. PubMed ID: 30341570
[TBL] [Abstract][Full Text] [Related]
16. Mice doubly-deficient in lysosomal hexosaminidase A and neuraminidase 4 show epileptic crises and rapid neuronal loss.
Seyrantepe V; Lema P; Caqueret A; Dridi L; Bel Hadj S; Carpentier S; Boucher F; Levade T; Carmant L; Gravel RA; Hamel E; Vachon P; Di Cristo G; Michaud JL; Morales CR; Pshezhetsky AV
PLoS Genet; 2010 Sep; 6(9):e1001118. PubMed ID: 20862357
[TBL] [Abstract][Full Text] [Related]
17. GM2 gangliosidoses in Spain: analysis of the HEXA and HEXB genes in 34 Tay-Sachs and 14 Sandhoff patients.
Gort L; de Olano N; Macías-Vidal J; Coll MA;
Gene; 2012 Sep; 506(1):25-30. PubMed ID: 22789865
[TBL] [Abstract][Full Text] [Related]
18. 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. PubMed ID: 7610760
[TBL] [Abstract][Full Text] [Related]
19. Highly phosphomannosylated enzyme replacement therapy for GM2 gangliosidosis.
Tsuji D; Akeboshi H; Matsuoka K; Yasuoka H; Miyasaki E; Kasahara Y; Kawashima I; Chiba Y; Jigami Y; Taki T; Sakuraba H; Itoh K
Ann Neurol; 2011 Apr; 69(4):691-701. PubMed ID: 21520232
[TBL] [Abstract][Full Text] [Related]
20. Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease.
Yamanaka S; Johnson MD; Grinberg A; Westphal H; Crawley JN; Taniike M; Suzuki K; Proia RL
Proc Natl Acad Sci U S A; 1994 Oct; 91(21):9975-9. PubMed ID: 7937929
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
