289 related articles for article (PubMed ID: 10757351)
1. Correction of acid beta-galactosidase deficiency in GM1 gangliosidosis human fibroblasts by retrovirus vector-mediated gene transfer: higher efficiency of release and cross-correction by the murine enzyme.
Sena-Esteves M; Camp SM; Alroy J; Breakefield XO; Kaye EM
Hum Gene Ther; 2000 Mar; 11(5):715-27. PubMed ID: 10757351
[TBL] [Abstract][Full Text] [Related]
2. Enzyme replacement for GM1-gangliosidosis: Uptake, lysosomal activation, and cellular disease correction using a novel β-galactosidase:RTB lectin fusion.
Condori J; Acosta W; Ayala J; Katta V; Flory A; Martin R; Radin J; Cramer CL; Radin DN
Mol Genet Metab; 2016 Feb; 117(2):199-209. PubMed ID: 26766614
[TBL] [Abstract][Full Text] [Related]
3. Impaired elastic-fiber assembly by fibroblasts from patients with either Morquio B disease or infantile GM1-gangliosidosis is linked to deficiency in the 67-kD spliced variant of beta-galactosidase.
Hinek A; Zhang S; Smith AC; Callahan JW
Am J Hum Genet; 2000 Jul; 67(1):23-36. PubMed ID: 10841810
[TBL] [Abstract][Full Text] [Related]
4. Retrovirus-mediated transfer and expression of beta-hexosaminidase alpha-chain cDNA in human fibroblasts from G(M2)-gangliosidosis B1 variant.
Teixeira CA; Sena-Esteves M; Lopes L; Sá Miranda MC; Ribeiro MG
Hum Gene Ther; 2001 Sep; 12(14):1771-83. PubMed ID: 11560770
[TBL] [Abstract][Full Text] [Related]
5. Transient high-level expression of beta-galactosidase after transfection of fibroblasts from GM1 gangliosidosis patients with plasmid DNA.
Balestrin RC; Baldo G; Vieira MB; Sano R; Coelho JC; Giugliani R; Matte U
Braz J Med Biol Res; 2008 Apr; 41(4):283-8. PubMed ID: 18392450
[TBL] [Abstract][Full Text] [Related]
6. Characterization of beta-galactosidase mutations Asp332-->Asn and Arg148-->Ser, and a polymorphism, Ser532-->Gly, in a case of GM1 gangliosidosis.
Zhang S; Bagshaw R; Hilson W; Oho Y; Hinek A; Clarke JT; Callahan JW
Biochem J; 2000 Jun; 348 Pt 3(Pt 3):621-32. PubMed ID: 10839995
[TBL] [Abstract][Full Text] [Related]
7. Complete correction of enzymatic deficiency and neurochemistry in the GM1-gangliosidosis mouse brain by neonatal adeno-associated virus-mediated gene delivery.
Broekman ML; Baek RC; Comer LA; Fernandez JL; Seyfried TN; Sena-Esteves M
Mol Ther; 2007 Jan; 15(1):30-7. PubMed ID: 17164772
[TBL] [Abstract][Full Text] [Related]
8. Mechanisms of distribution of mouse beta-galactosidase in the adult GM1-gangliosidosis brain.
Broekman ML; Tierney LA; Benn C; Chawla P; Cha JH; Sena-Esteves M
Gene Ther; 2009 Feb; 16(2):303-8. PubMed ID: 18818671
[TBL] [Abstract][Full Text] [Related]
9. Intracellular processing and maturation of mutant gene products in hereditary beta-galactosidase deficiency (beta-galactosidosis).
Oshima A; Yoshida K; Itoh K; Kase R; Sakuraba H; Suzuki Y
Hum Genet; 1994 Feb; 93(2):109-14. PubMed ID: 8112731
[TBL] [Abstract][Full Text] [Related]
10. Chaperone therapy update: Fabry disease, GM1-gangliosidosis and Gaucher disease.
Suzuki Y
Brain Dev; 2013 Jun; 35(6):515-23. PubMed ID: 23290321
[TBL] [Abstract][Full Text] [Related]
11. [beta-galactosidosis--GM1 gangliosidosis and Morquio B disease].
Yoshida K; Yanagisawa N
Nihon Rinsho; 1995 Dec; 53(12):2960-6. PubMed ID: 8577043
[TBL] [Abstract][Full Text] [Related]
12. Molecular basis of GM1 gangliosidosis and Morquio disease, type B. Structure-function studies of lysosomal beta-galactosidase and the non-lysosomal beta-galactosidase-like protein.
Callahan JW
Biochim Biophys Acta; 1999 Oct; 1455(2-3):85-103. PubMed ID: 10571006
[TBL] [Abstract][Full Text] [Related]
13. Generation and characterization of recombinant feline beta-galactosidase for preclinical enzyme replacement therapy studies in GM1 gangliosidosis.
Samoylova TI; Martin DR; Morrison NE; Hwang M; Cochran AM; Samoylov AM; Baker HJ; Cox NR
Metab Brain Dis; 2008 Jun; 23(2):161-73. PubMed ID: 18421424
[TBL] [Abstract][Full Text] [Related]
14. (5aR)-5a-C-Pentyl-4-epi-isofagomine: A powerful inhibitor of lysosomal β-galactosidase and a remarkable chaperone for mutations associated with GM1-gangliosidosis and Morquio disease type B.
Front S; Biela-Banaś A; Burda P; Ballhausen D; Higaki K; Caciotti A; Morrone A; Charollais-Thoenig J; Gallienne E; Demotz S; Martin OR
Eur J Med Chem; 2017 Jan; 126():160-170. PubMed ID: 27750150
[TBL] [Abstract][Full Text] [Related]
15. Characteristics of the beta-galactosidase-carboxypeptidase complex in GM1-gangliosidosis and beta-galactosialidosis fibroblasts.
D'Agrosa RM; Hubbes M; Zhang S; Shankaran R; Callahan JW
Biochem J; 1992 Aug; 285 ( Pt 3)(Pt 3):833-8. PubMed ID: 1497621
[TBL] [Abstract][Full Text] [Related]
16. The potential action of galactose as a "chemical chaperone": increase of beta galactosidase activity in fibroblasts from an adult GM1-gangliosidosis patient.
Caciotti A; Donati MA; d'Azzo A; Salvioli R; Guerrini R; Zammarchi E; Morrone A
Eur J Paediatr Neurol; 2009 Mar; 13(2):160-4. PubMed ID: 18571950
[TBL] [Abstract][Full Text] [Related]
17. Biochemical and molecular characterization of novel mutations in GLB1 and NEU1 in patient cells with lysosomal storage disorders.
Kwak JE; Son MY; Son YS; Son MJ; Cho YS
Biochem Biophys Res Commun; 2015 Feb; 457(4):554-60. PubMed ID: 25600812
[TBL] [Abstract][Full Text] [Related]
18. Chemical chaperone therapy: chaperone effect on mutant enzyme and cellular pathophysiology in β-galactosidase deficiency.
Higaki K; Li L; Bahrudin U; Okuzawa S; Takamuram A; Yamamoto K; Adachi K; Paraguison RC; Takai T; Ikehata H; Tominaga L; Hisatome I; Iida M; Ogawa S; Matsuda J; Ninomiya H; Sakakibara Y; Ohno K; Suzuki Y; Nanba E
Hum Mutat; 2011 Jul; 32(7):843-52. PubMed ID: 21520340
[TBL] [Abstract][Full Text] [Related]
19. Normal serum beta-galactosidase in juvenile GM1 gangliosidosis.
Ishii N; Oshima A; Sakuraba H; Fukuyama Y; Suzuki Y
Pediatr Neurol; 1994 Jun; 10(4):317-9. PubMed ID: 8068159
[TBL] [Abstract][Full Text] [Related]
20. Molecular form and subcellular distribution of acid beta-galactosidase in fibroblasts from patients with GM1 gangliosidosis, Morquio B disease and galactosialidosis.
Takiyama N; Itoh K; Shimmoto M; Nishimoto J; Inui K; Sakuraba H; Suzuki Y
Brain Dev; 1997 Mar; 19(2):126-30. PubMed ID: 9105659
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
