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1 odegenerative lysosomal storage disease, GM1 gangliosidosis.
2 ncy of beta-Gal augmentation therapy for GM1 gangliosidosis.
3 eta galactosidase-1, which is mutated in GM1 gangliosidosis.
4 odegenerative lysosomal storage disease, GM1 gangliosidosis.
5 regions of pathology in a mouse model of GM1 gangliosidosis.
6 odegenerative lysosomal storage disease, GM1 gangliosidosis.
7  type II, mucopolysacharidosis type III, GM1 gangliosidosis.
8  a mouse model of Sandhoff disease, a lethal gangliosidosis.
9 neuronal apoptosis in the mouse model of GM1-gangliosidosis.
10 of Tay-Sachs disease known as variant AB GM2 gangliosidosis.
11 regions of pathology in a mouse model of GM1 gangliosidosis.
12 lhexosaminidase, the enzyme deficient in GM2 gangliosidosis.
13 d that PGRN deficiency in lysosomes leads to gangliosidosis.
14 erapy as a safe, effective treatment for GM1 gangliosidosis.
15 e natural history of adult patients with GM2 gangliosidosis.
16                  One such condition is G(M1)-gangliosidosis, a neurodegenerative glycosphingolipidosi
17 of the cardinal pathological features of GM2 gangliosidosis, a point is reached when functional deter
18 roblasts but not from fibroblasts of a G(M1) gangliosidosis and a galactosialidosis patient.
19 brain for PLD3; spleen for PLD4), leading to gangliosidosis and lysosomal abnormalities.
20 t in the autosomal recessive disorders G(M1) gangliosidosis and Morquio B, is synthesized as an 85-kD
21 the nature of the neurological injury in GM2 gangliosidosis and the extent of its reversibility, we h
22 acid beta-galactosidase knockout mice (G(M1) gangliosidosis) and found that the acid beta-galactosida
23 ed neuron-specific Hexb production, reversed gangliosidosis, and ameliorated peripheral sensory dysfu
24 ning NEU1 at physiological levels in the GM1 gangliosidosis brain.
25 racterizes the neurodegenerative disease GM1-gangliosidosis, but whether the accumulation of GM1 is d
26 ortest lifespan, despite the fact that G(M1) gangliosidosis carrier mice with an otherwise normal gen
27                      Sandhoff disease, a GM2 gangliosidosis caused by a deficiency in beta-hexosamini
28                 In its most severe form, GM1 gangliosidosis causes death by 4 years of age, and no ef
29 l in lysosomal storage diseases (GM1 and GM2 gangliosidosis; Fabry, Gaucher, and Krabbe diseases; and
30                                          GM1 gangliosidosis (GM1) is an autosomal recessive lysosomal
31 l of the human lysosomal storage disease GM1-gangliosidosis, GM1-ganglioside accumulates in the glyco
32 asts from patients with Fabry's disease, GM1 gangliosidosis, GM2 gangliosidosis (Tay-Sachs and Sandho
33 tics resembling those noted in infantile GM2 gangliosidosis has been described.
34                  First reported in 1881, GM2 gangliosidosis has no effective treatment today, and chi
35 te onset Tay-Sachs (LOTS), Sandhoff] and GM1 gangliosidosis have been studied to determine whether th
36                    Improved knowledge of GM2 gangliosidosis in adults will help clinicians achieve co
37 e model of Tay-Sachs disease, a severe human gangliosidosis, indicating that this enzyme is responsib
38                                          GM1 gangliosidosis is a fatal neurodegenerative disease caus
39                                    Human GM1-gangliosidosis is caused by a genetic deficiency of lyso
40 roimaging which are initial findings for GM2 gangliosidosis is important from the point of diagnosis
41                                          GM2 gangliosidosis is usually fatal by 5 years of age in its
42 read expression of betagal in the CNS of GM1 gangliosidosis mice is sufficient to achieve significant
43 rs and delivery of mod2B to lysosomes in GM2 gangliosidosis models.
44 eta-Gal, NEU1 levels are elevated in the GM1 gangliosidosis mouse brain, which are restored to normal
45 activity and facilitated its recovery in GM1 gangliosidosis patient fibroblasts by 2-6-fold.
46 iral-mediated GLB1 overexpression in the GM1 gangliosidosis patient fibroblasts caused accumulation o
47 irus-mediated GLB1 overexpression in the GM1 gangliosidosis patient fibroblasts caused accumulation o
48 ntiviral-mediated GLB1 overexpression in GM1 gangliosidosis patient fibroblasts coincides with profou
49 t to augment beta-Gal activity levels in GM1 gangliosidosis patient fibroblasts without promoting NEU
50  give new significance to mutations in G(M1) gangliosidosis patients found in the C-terminal part of
51 erapy (ERT) approach in fibroblasts from GM1 gangliosidosis patients with recombinant human beta-gal
52 erapy (ERT) approach in fibroblasts from GM1 gangliosidosis patients with recombinant human Beta-Gal
53 ve therapeutic intervention for managing GM1 gangliosidosis potentially more safely than with gene th
54 the frontal cortices of postmortem human GM1 gangliosidosis, Sandhoff disease, and Tay-Sachs disease
55  proteins are also enriched at the PM in GM1 gangliosidosis supporting that lysosomal exocytosis is a
56 ith Fabry's disease, GM1 gangliosidosis, GM2 gangliosidosis (Tay-Sachs and Sandhoff forms), metachrom
57 id and rescue phenotypic consequences of GM2 gangliosidosis, Tay-Sachs and Sandhoff diseases in anima
58 is, globoid cell leukodystrophy, GM1 and GM2 gangliosidosis, the mucopolysaccharidoses, and neuronal
59       Using an authentic murine model of GM2 gangliosidosis, we examined the pattern of neuronal loss
60      Thus, a disease condition such as G(M1)-gangliosidosis, which is characterized by neurodegenerat