نتایج جستجو برای: گانگلیوزیدوز gm2

تعداد نتایج: 703  

Journal: :Human gene therapy 2016
Subha Karumuthil-Melethil Sahana Nagabhushan Kalburgi Patrick Thompson Michael Tropak Michael D Kaytor John G Keimel Brian L Mark Don Mahuran Jagdeep S Walia Steven J Gray

GM2 gangliosidosis is a family of three genetic neurodegenerative disorders caused by the accumulation of GM2 ganglioside (GM2) in neuronal tissue. Two of these are due to the deficiency of the heterodimeric (α-β), "A" isoenzyme of lysosomal β-hexosaminidase (HexA). Mutations in the α-subunit (encoded by HEXA) lead to Tay-Sachs disease (TSD), whereas mutations in the β-subunit (encoded by HEXB)...

Journal: :Pediatric Neurology Briefs 2006

Journal: :Cancer research 1986
E J Natoli P O Livingston C S Pukel K O Lloyd H Wiegandt J Szalay H F Oettgen L J Old

Antisera reactive with the ganglioside GM2 were raised by immunizing C57BL/6 mice with the C57BL/6 melanoma JB-RH. Fusion with NS-1 was performed using splenic mononuclear cells from a mouse with high antibody titer. An immunoglobulin M monoclonal antibody (monoclonal antibody 5-3) was identified which was reactive with an antigen that was resistant to heat, trypsin, and Pronase. A panel of pur...

Journal: :Journal of lipid research 2014
Martin Gantner Günter Schwarzmann Konrad Sandhoff Thomas Kolter

Within recent years, ganglioside patterns have been increasingly analyzed by MS. However, internal standards for calibration are only available for gangliosides GM1, GM2, and GM3. For this reason, we prepared homologous internal standards bearing nonnatural fatty acids of the major mammalian brain gangliosides GM1, GD1a, GD1b, GT1b, and GQ1b, and of the tumor-associated gangliosides GM2 and GD2...

Journal: :Clinical cancer research : an official journal of the American Association for Cancer Research 1998
S Zhang H S Zhang V E Reuter S F Slovin H I Scher P O Livingston

Defining the expression of tumor-associated antigens on primary and metastatic prostate cancer is the crucial first step in selecting appropriate targets for immune attack. In this study, the distribution of the tumor-associated antigens GM2, Tn, sTn, Thompson-Friedenreich antigen (TF), Globo H, Le(y), MUC1, MUC2, MUC3, MUC4, MUC5AC, MUC5B, MUC7, carcinoembryonic antigen, beta chain of human ch...

2012
Timothy J. Sargeant Deborah J. Drage Susan Wang Apostolos A. Apostolakis Timothy M. Cox M. Begoña Cachón-González

Tay-Sachs and Sandhoff diseases are lethal inborn errors of acid β-N-acetylhexosaminidase activity, characterized by lysosomal storage of GM2 ganglioside and related glycoconjugates in the nervous system. The molecular events that lead to irreversible neuronal injury accompanied by gliosis are unknown; but gene transfer, when undertaken before neurological signs are manifest, effectively rescue...

2015
Jennifer Stencel-Baerenwald Kerstin Reiss Bärbel S. Blaum Daniel Colvin Xiao-Nan Li Ty Abel Kelli Boyd Thilo Stehle Terence S. Dermody

UNLABELLED Receptors expressed on the host cell surface adhere viruses to target cells and serve as determinants of viral tropism. Several viruses bind cell surface glycans to facilitate entry, but the contribution of specific glycan moieties to viral disease is incompletely understood. Reovirus provides a tractable experimental model for studies of viral neuropathogenesis. In newborn mice, ser...

Journal: :Cancer research 2006
Kaushik Biswas Amy Richmond Patricia Rayman Soumika Biswas Mark Thornton Gaurisankar Sa Tanya Das Renliang Zhang Ali Chahlavi Charles S Tannenbaum Andrew Novick Ronald Bukowski James H Finke

Multiple mechanisms have been proposed to account for immune escape by tumors. Although gangliosides have long been known to suppress T-cell immunity, few studies have examined the effect of human tumor-derived gangliosides on immune responses. Here, we show that gangliosides isolated from renal cell carcinoma (RCC) cell lines and clear cell tumor tissue can induce apoptosis in peripheral blood...

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