نتایج جستجو برای: dimethyl wp48 percent at one gm2
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GM2-gangliosidosis, AB variant is an extremely rare autosomal recessive inherited disorder caused by mutations in the GM2A gene that encodes GM2 ganglioside activator protein (GM2AP). GM2AP is necessary for solubilisation of GM2 ganglioside in endolysosomes and its presentation to β-hexosaminidase A. Conversely GM2AP deficiency impairs lysosomal catabolism of GM2 ganglioside, leading to its sto...
the present study aims at identifying, classifying and analyzing collocation errors made by translators of the holy quran into english.findings indicated that collocationally the most acceptablt translation was done by ivring but the least appropriate one made by pickthall.
the present study sought to investigate the role of dynamic assessment (da) in improvement of iranian efl writing performance at different language proficiency levels. to this end, after conducting the quick placement test, 60 iranian efl learners were assigned to two groups with different language proficiency levels. in both groups each participant wrote two compositions, one before and one af...
Our previous studies have shown accumulation of GM2 ganglioside during ethanol-induced neurodegeneration in the developing brain, and GM2 elevation has also been reported in other brain injuries and neurodegenerative diseases. Using GM2/GD2 synthase KO mice lacking GM2/GD2 and downstream gangliosides, the current study explored the significance of GM2 elevation in WT mice. Immunohistochemical s...
Complex ganglioside expression is highly deregulated in several tumors which is further dependent on specific ganglioside synthase genes. Here, we designed and constructed a pair of highly specific transcription-activator like effector endonuclease (TALENs) to disrupt a particular genomic locus of mouse GM2-synthase, a region conserved in coding sequence of all four transcript variants of mouse...
To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex) B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-...
The GM2 activator deficiency (also known as the AB variant), Tay-Sachs disease, and Sandhoff disease are the major forms of the GM2 gangliosidoses, disorders caused by defective degradation of GM2 ganglioside. Tay-Sachs and Sandhoff diseases are caused by mutations in the genes (HEXA and HEXB) encoding the subunits of beta-hexosaminidase A. The GM2 activator deficiency is caused by mutations in...
بررسی رابطه بین یادگیری سازمانی و توانمندسازی مدیران و معاونین مدارس شهرستان میانه دکتر محمد اورکی1، دکتر هاشم نعمتی، گلسیما عزیزی* استادیار گروه علوم تربیتی ، دانشگاه پیام نور تهران جنوب استادیار گروه علوم تربیتی. ، دانشگاه پیام نور مشهد دانشجوی کارشناسی ارشد مدیریت آموزشی ، دانشگاه پیام نور تهران جنوب [email protected] میانه- دانشگاه پیام نور میانه مدیر آموزشی، 2226062 – 0423، ت...
GM2 gangliosidoses are autosomal recessive lysosomal storage diseases (LSDs) caused by mutations in the HEXA, HEXB and GM2A genes, which encode the human lysosomal β-hexosaminidase (Hex) α- and β-subunits, and GM2 activator protein (GM2A), respectively. These diseases are associated with excessive accumulation of GM2 ganglioside (GM2) in the brains of patients with neurological symptoms. Here w...
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