328 related articles for article (PubMed ID: 31896760)
41. Functionality of a bicistronic construction containing
Shaimardanova AA; Chulpanova DS; Solovyeva VV; Aimaletdinov AM; Rizvanov AA
Neural Regen Res; 2022 Jan; 17(1):122-129. PubMed ID: 34100447
[TBL] [Abstract][Full Text] [Related]
42. 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. PubMed ID: 23483939
[TBL] [Abstract][Full Text] [Related]
43. Biochemical consequences of mutations causing the GM2 gangliosidoses.
Mahuran DJ
Biochim Biophys Acta; 1999 Oct; 1455(2-3):105-38. PubMed ID: 10571007
[TBL] [Abstract][Full Text] [Related]
44. 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; 49(3):834-40. PubMed ID: 3612128
[TBL] [Abstract][Full Text] [Related]
45. 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]
46. Epstein-Barr virus transformed lymphoid cell lines as a new model system in culture for the study of GM2-gangliosidoses: Tay-Sachs and Sandhoff diseases.
Maret A; Salvayre R; Negre A; Bes JC; Douste-Blazy L
Biol Cell; 1985; 53(3):293-6. PubMed ID: 2990625
[TBL] [Abstract][Full Text] [Related]
47. Two small deletion mutations of the HEXB gene are present in DNA from a patient with infantile Sandhoff disease.
McInnes B; Brown CA; Mahuran DJ
Biochim Biophys Acta; 1992 Apr; 1138(4):315-7. PubMed ID: 1532910
[TBL] [Abstract][Full Text] [Related]
48. Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease.
Mark BL; Mahuran DJ; Cherney MM; Zhao D; Knapp S; James MN
J Mol Biol; 2003 Apr; 327(5):1093-109. PubMed ID: 12662933
[TBL] [Abstract][Full Text] [Related]
49. Cerebral organoids derived from Sandhoff disease-induced pluripotent stem cells exhibit impaired neurodifferentiation.
Allende ML; Cook EK; Larman BC; Nugent A; Brady JM; Golebiowski D; Sena-Esteves M; Tifft CJ; Proia RL
J Lipid Res; 2018 Mar; 59(3):550-563. PubMed ID: 29358305
[TBL] [Abstract][Full Text] [Related]
50. Increased phosphorylation of HexM improves lysosomal uptake and potential for managing GM2 gangliosidoses.
Benzie G; Bouma K; Battellino T; Cooper S; Hemming R; Kammouni W; Liu L; Do C; Khajehpour M; Perreault H; Kornfeld S; Triggs-Raine B; Mark BL
BBA Adv; 2022; 2():100032. PubMed ID: 37082581
[TBL] [Abstract][Full Text] [Related]
51. Glycosphingolipid degradation and animal models of GM2-gangliosidoses.
Kolter T; Sandhoff K
J Inherit Metab Dis; 1998 Aug; 21(5):548-63. PubMed ID: 9728335
[TBL] [Abstract][Full Text] [Related]
52. 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]
53. 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]
54. Tay-Sachs and Sandhoff diseases: enzymatic diagnosis in dried blood spots on filter paper: retrospective diagnoses in newborn-screening cards.
Chamoles NA; Blanco M; Gaggioli D; Casentini C
Clin Chim Acta; 2002 Apr; 318(1-2):133-7. PubMed ID: 11880123
[TBL] [Abstract][Full Text] [Related]
55. Imaging MALDI mass spectrometry using an oscillating capillary nebulizer matrix coating system and its application to analysis of lipids in brain from a mouse model of Tay-Sachs/Sandhoff disease.
Chen Y; Allegood J; Liu Y; Wang E; Cachón-Gonzalez B; Cox TM; Merrill AH; Sullards MC
Anal Chem; 2008 Apr; 80(8):2780-8. PubMed ID: 18314967
[TBL] [Abstract][Full Text] [Related]
56. Introduction of an N-glycan sequon into HEXA enhances human beta-hexosaminidase cellular uptake in a model of Sandhoff disease.
Matsuoka K; Tsuji D; Aikawa S; Matsuzawa F; Sakuraba H; Itoh K
Mol Ther; 2010 Aug; 18(8):1519-26. PubMed ID: 20571546
[TBL] [Abstract][Full Text] [Related]
57. 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]
58. [Tay-Sachs disease].
Tanaka A
Nihon Rinsho; 1993 Sep; 51(9):2281-5. PubMed ID: 8411703
[TBL] [Abstract][Full Text] [Related]
59. [Genetic study of GM2 gangliosidosis (Tay-Sachs and Sandhoff) by the study of the hexosaminidases of the Sandhoff-rodents hybrids (mouse and hamster)].
Weil D; Van Cong N; Rebourcet R; Frézal J
Ann Genet; 1975 Sep; 18(3):163-8. PubMed ID: 810067
[TBL] [Abstract][Full Text] [Related]
60. Combined replacement effects of human modified β-hexosaminidase B and GM2 activator protein on GM2 gangliosidoses fibroblasts.
Kitakaze K; Tasaki C; Tajima Y; Hirokawa T; Tsuji D; Sakuraba H; Itoh K
Biochem Biophys Rep; 2016 Sep; 7():157-163. PubMed ID: 28955902
[TBL] [Abstract][Full Text] [Related]
[Previous] [Next] [New Search]
