نتایج جستجو برای: sandhoff disease
تعداد نتایج: 1490121 فیلتر نتایج به سال:
Saposins (A, B, C, and D) are small glycoproteins required for the hydrolysis of sphingolipids by specific lysosomal hydrolases. Concentrations of these saposins in brain, liver, and spleen from normal humans as well as patients with lysosomal storage disease were determined. A quantitative HPLC method was used for saposin A, C, and D and a stimulation assay was used for saposin B. In normal ti...
Sandhoff disease (SD) is a glycosphingolipid storage disease that arises from mutations in the Hexb gene and the resultant deficiency in β-hexosaminidase activity. This deficiency results in aberrant lysosomal accumulation of the ganglioside GM2 and related glycolipids, and progressive deterioration of the central nervous system. Dysfunctional glycolipid storage causes severe neurodegeneration ...
Caloric restriction (CR), which improves health and increases longevity, was studied as a therapy in a hexosaminidase beta knockout mouse model of Sandhoff disease (SD), an incurable neurodegenerative disease involving accumulation of brain ganglioside GM2 and asialo-GM2 (GA2). Adult mice were fed a rodent chow diet either ad libitum (AL) or restricted to reduce body weight by 15-18% (CR). Alth...
13961400 8. Quiroga, S., Caputto, B. I,. & Caputto, R. (1984) J. Neurosci. Res. 12,269-273 9. Conzelmann, E. & Sandhoff, K. (1979) HoppeSeyler’s Z. Physiol. Chem. 360, 1837-1849 10. Caputto, H. I,., Nores, G. A., Cemborain, H. N. & Caputto, R. (1982) Brain Res. 245,231-238 11. Chatterjee, S., Clarke, K. S. & Kwiterovich Jr., 1’. 0 . (1986) J. Hiol. Chem. 261, 13474-13479 12. Chatterjee, S., Gho...
نمودار تعداد نتایج جستجو در هر سال
با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید