戻る
「早戻しボタン」を押すと検索画面に戻ります。 [閉じる]

コーパス検索結果 (１語後でソート)

通し番号をクリックするとPubMedの該当ページを表示します
1 -deficient mice, a model of the neuronopathy Sandhoff disease.
2 rains from this mouse model of Tay-Sachs and Sandhoff disease.
3 storage diseases: NPC, mucolipidosis IV, and Sandhoff disease.
4 ivation also was detected in a human case of Sandhoff disease.
5 f age in its 2 major subtypes, Tay-Sachs and Sandhoff disease.
6 tional GM2 accumulates causing Tay-Sachs and Sandhoff diseases.
7                                              Sandhoff disease, a GM2 gangliosidosis caused by a defic
8 y and prolonged survival in a mouse model of Sandhoff disease, a lethal gangliosidosis.
9 eplacement method rescued the mouse model of Sandhoff disease, a lysosomal storage disease caused by
10  in neurodegeneration using a mouse model of Sandhoff disease, a prototypical neuronopathic lysosomal
11 ices of postmortem human GM1 gangliosidosis, Sandhoff disease, and Tay-Sachs disease brains.
12 e conditions that include Tay-Sachs disease, Sandhoff disease, and the GM2 activator deficiency.
13                                           In Sandhoff disease, another sphingolipid disorder, neuroin
14 n as the AB variant), Tay-Sachs disease, and Sandhoff disease are the major forms of the GM2 ganglios
15                                Tay-Sachs and Sandhoff diseases are caused by mutations in the genes (
16                                Tay-Sachs and Sandhoff diseases are lysosomal storage disorders charac
17        The GM2 gangliosidoses, Tay-Sachs and Sandhoff diseases, are caused by mutations in the HEXA (
18  GM2 gangliosidoses, including Tay-Sachs and Sandhoff diseases, are neurodegenerative lysosomal stora
19 ivery system the dysfunction of which drives Sandhoff disease, but which can be corrected by hematopo
20  effectively treats neuropathology in feline Sandhoff disease, but whole-body targeting will be an im
21          GINI was tested on colon cancer and Sandhoff disease cell lines, which contained previously
22 olipid storage diseases, NPC2 deficiency and Sandhoff disease, characterized by sphingolipid traffick
23 quences of GM2 gangliosidosis, Tay-Sachs and Sandhoff diseases in animal models following simultaneou
24 he results indicate that the pathogenesis of Sandhoff disease involves an increase in MIP-1alpha that
25                                              Sandhoff disease is a lysosomal storage disorder charact
26                                              Sandhoff disease is a prototypical lysosomal storage dis
27                                              Sandhoff disease is an autosomal recessive disorder caus
28  that their lack of storage in Tay-Sachs and Sandhoff diseases is due to functional redundancy in the
29 oth mice and patients with neurodegenerative Sandhoff disease leads to a massive accumulation of GM2
30                    While untreated cats with Sandhoff disease lived for 4.4 +/- 0.6 months, AAV-treat
31 crophage/microglial population in the CNS of Sandhoff disease mice is compounded by the infiltration
32               Bone marrow transplantation of Sandhoff disease mice suppressed both the explosive expa
33  sphingosine kinase 1 (Sphk1) was deleted in Sandhoff disease mice, a milder disease course occurred,
34                               In symptomatic Sandhoff disease mice, apoptotic neuronal cell death was
35 otor dysfunction and a prolonged lifespan in Sandhoff disease mice.
36  the terminal stages of neurodegeneration in Sandhoff disease mice.
37 athology together with neuronal apoptosis in Sandhoff disease mice.
38 ganglioside accumulation in a mouse model of Sandhoff disease might be associated with the accumulati
39 is phenotype is not seen in the Tay-Sachs or Sandhoff disease model mice or in the corresponding huma
40 etry to monitor efficacy of this approach in Sandhoff disease model mice.
41 ount of beta-hexosaminidase S present in the Sandhoff disease model mice.
42 ential treatment for these disorders using a Sandhoff disease mouse model.
43  cerebral cortex from a Tay-Sachs patient, a Sandhoff disease patient and a pediatric control.
44                                              Sandhoff disease (SD) is an LSD caused by a deficiency i
45                  An authentic mouse model of Sandhoff disease (SD) with pathological characteristics
46 n the HEXA (Tay-Sachs disease [TS]) or HEXB (Sandhoff disease [SD]) genes, with subsequent low hexosa
47 ecal infusion in children with Tay-Sachs and Sandhoff diseases (six infantile, three juvenile).
48 ents a 22-month-old girl with infantile type Sandhoff disease that was hospitalized for generalized s
49 exb gene (hexb-/-), a model of Tay-Sachs and Sandhoff disease, versus the functionally normal heteroz
50                                    Cats with Sandhoff disease were treated by intracranial injection