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8. GM2-ganglioside metabolism in hexosaminidase A deficiency states: determination in situ using labeled GM2 added to fibroblast cultures. Raghavan SS; Krusell A; Krusell J; Lyerla TA; Kolodny EH Am J Hum Genet; 1985 Nov; 37(6):1071-82. PubMed ID: 2934978 [TBL] [Abstract][Full Text] [Related]
9. Ganglioside loading of cultured fibroblasts: a provocative method for the diagnosis of the GM2 gangliosidoses. Charrow J; Binns HJ Clin Chim Acta; 1986 Apr; 156(1):41-9. PubMed ID: 2938852 [TBL] [Abstract][Full Text] [Related]
10. 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. PubMed ID: 21487393 [TBL] [Abstract][Full Text] [Related]
11. Assay of the GM2-ganglioside cleaving hexosaminidase activity of skin fibroblasts for GM2-gangliosidoses. Harzer K Clin Chim Acta; 1983 Nov; 135(1):89-93. PubMed ID: 6228344 [No Abstract] [Full Text] [Related]
12. Juvenile GM2 gangliosidosis (AMB variant): inability to activate hexosaminidase A by activator protein. Inui K; Grebner EE; Jackson LG; Wenger DA Am J Hum Genet; 1983 Jul; 35(4):551-64. PubMed ID: 6224417 [TBL] [Abstract][Full Text] [Related]
13. 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. PubMed ID: 8875224 [TBL] [Abstract][Full Text] [Related]
14. 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]
15. The human GM2 activator protein. A substrate specific cofactor of beta-hexosaminidase A. Meier EM; Schwarzmann G; Fürst W; Sandhoff K J Biol Chem; 1991 Jan; 266(3):1879-87. PubMed ID: 1824846 [TBL] [Abstract][Full Text] [Related]
16. Cytology, growth characteristics and cellular alterations following SV40-induced transformation of human foetal brain cells derived from a Gm2 gangliosidosis and control. Brooks SE; Amsterdam D; Hoffman LM; Adachi M; Schneck L J Cell Sci; 1979 Aug; 38():211-23. PubMed ID: 230195 [TBL] [Abstract][Full Text] [Related]
17. Hereditary heat-labile hexosaminidase B: a variant whose homozygotes synthesize a functional HEX A. Navon R; Kopel R; Nutman J; Frisch A; Conzelmann E; Sandhoff K; Adam A Am J Hum Genet; 1985 Jan; 37(1):138-46. PubMed ID: 3156493 [TBL] [Abstract][Full Text] [Related]