Gangliosidoses Gm1
Mostrando 1-5 de 5 artigos, teses e dissertações.
-
1. Comparison of the effect of ganglioside GM1 and the Nerve Growth Factor (NGF) on the expression of receiver of high affinity for NGF, TrkA and insulin in isolated pancreatic islets of NOD mice (non obese diabetic) / Comparação dos efeitos do gangliosideo GM1 e do fator de crescimento neural (NGF) sobre a expressão de receptor de alta afinidade para NGF, TrkA e insulina em ilhotas pancreaticas isoladas de camundongos NOD (diabetico não obeso)
The non-obese diabetic mice (NOD) lineage is characterized by developing type 1 diabetes mellitus (DM-1) naturally, bearing a similarity to DM-1 in human beings. The spontaneous manifestation of diabetes is characterized by gradual infiltration in pancreatic islets by mononuclear cells lymphocytes T (CD4+ and CD8+) and destruction of the ß-cells producers o
Publicado em: 2008
-
2. Triagem de novas mutações em pacientes com gangliosidose GM1 através da análise do polimorfismo conformacional de fita simples de DNA (SSCP)
Publicado em: 2007
-
3. Effect of GM1 ganglioside in the sciatic nerves of the NOD (non obse diabetic) / Estudo do efeito do gangliosideo GM1 sobre os nervos perifericos do camundongo NOD (Non Obese Diabetic)
A linhagem de camundongos NOD (non obese diabetic) desenvolve espontaneamente diabetes mellitus tipo 1 (DM-1) com marcante similaridade ao observado em humanos, que se estabelece entre 12ª e 24ª semana de vida. Os gangliosideos são glicoesfingolipídeos de membrana que contém ácido siálico em sua composição e estão presentes na maioria das células
Publicado em: 2007
-
4. Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease.
Tay-Sachs disease, the prototype of the GM2 gangliosidoses, is a catastrophic neurodegenerative disorder of infancy. The disease is caused by mutations in the HEXA gene resulting in an absence of the lysosomal enzyme, beta-hexosaminidase A. As a consequence of the enzyme deficiency, GM2 ganglioside accumulates progressively, beginning early in fetal life, to
-
5. Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin
Sandhoff disease is a neurodegenerative disorder resulting from the autosomal recessive inheritance of mutations in the HEXB gene, which encodes the β-subunit of β-hexosaminidase. GM2 ganglioside fails to be degraded and accumulates within lysosomes in cells of the periphery and the central nervous system (CNS). There are currently no therapies for the gly
The National Academy of Sciences.