selected scholarly activity
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academic article
- Suppression of NK and CD8+ T cells reduces astrogliosis but accelerates cerebellar dysfunction and shortens life span in a mouse model of Sandhoff disease.. Journal of Neuroimmunology. 306:55-67. 2017
- Asialoglycoprotein receptors as important mediators of plasma lipids and atherosclerosis. Current Opinion in Lipidology. 28:209-212. 2017
- Neuronal pentraxin 1 depletion delays neurodegeneration and extends life in Sandhoff disease mice.. Human Molecular Genetics. 26:661-673. 2017
- Bi-phasic gliosis drives neuropathology in a Sandhoff disease mouse model. Journal of Neuroimmunology. 299:19-27. 2016
- Characterization of Proliferating Lesion-Resident Cells During All Stages of Atherosclerotic Growth.. Journal of the American Heart Association. 5. 2016
- The effects of diet on occlusive coronary artery atherosclerosis and myocardial infarction in scavenger receptor class B, type 1/low-density lipoprotein receptor double knockout mice.. Arteriosclerosis, Thrombosis, and Vascular Biology. 34:2394-2403. 2014
- The effect of pomegranate extract on coronary artery atherosclerosis in SR-BI/APOE double knockout mice.. Atherosclerosis. 228:80-89. 2013
- SR-BI in bone marrow derived cells protects mice from diet induced coronary artery atherosclerosis and myocardial infarction.. PLoS ONE. 8:e72492. 2013
- Transient neonatal hyperparathyroidism: a presenting feature of sialidosis type II.. Journal of Pediatric Endocrinology and Metabolism. 26:767-769. 2013
- Hypomorphic sialidase expression decreases serum cholesterol by downregulation of VLDL production in mice.. Journal of Lipid Research. 53:2573-2585. 2012
- Miglustat as a therapeutic agent: prospects and caveats. Journal of Medical Genetics. 49:591-597. 2012
- The therapeutic potential of pharmacological chaperones and proteosomal inhibitors, Celastrol and MG132 in the treatment of sialidosis. Molecular Genetics and Metabolism. 107:173-185. 2012
- A point mutation in the neu1 promoter recruits an ectopic repressor, Nkx3.2 and results in a mouse model of sialidase deficiency.. Molecular Genetics and Metabolism. 97:43-52. 2009
- The inhibition of endocytosis affects HDL-lipid uptake mediated by the human scavenger receptor class B type I.. Molecular Membrane Biology. 24:442-454. 2007
- Regulation of SR-BI-mediated selective lipid uptake in Chinese hamster ovary-derived cells by protein kinase signaling pathways.. Journal of Lipid Research. 48:405-416. 2007
- Late onset Tay-Sachs disease in mice with targeted disruption of the Hexa gene: behavioral changes and pathology of the central nervous system.. Brain Research Brain Research Protocols Molecular Brain Research. 1001:37-50. 2004
- Functional rescue of vitamin C synthesis deficiency in human cells using adenoviral-based expression of murine l-gulono-gamma-lactone oxidase.. Genomics. 83:482-492. 2004
- Five novel mutations in the lysosomal sialidase gene (NEU1) in type II sialidosis patients and assessment of their impact on enzyme activity and intracellular targeting using adenovirus-mediated expression.. Human Mutation. 23:32-39. 2004
- Cathepsin A is expressed in a cell- and region-specific manner in the testis and epididymis and is not regulated by testicular or pituitary factors.. Journal of Histochemistry and Cytochemistry. 48:1131-1146. 2000
- Role of sphingolipids in the transport of prosaposin to the lysosomes.. Journal of Lipid Research. 40:1593-1603. 1999
- Sialidase-mediated depletion of GM2 ganglioside in Tay-Sachs neuroglia cells. Human Molecular Genetics. 8:1111-1116. 1999
- Cloning of the cDNA and Gene Encoding Mouse Lysosomal Sialidase and Correction of Sialidase Deficiency in Human Sialidosis and Mouse SM/J Fibroblasts. Human Molecular Genetics. 7:115-120. 1998
- Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases.. Human Molecular Genetics. 6:1879-1885. 1997
- Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis.. Nature Genetics. 15:316-320. 1997
- The Saccharomyces cerevisiae processing alpha 1,2-mannosidase is localized in the endoplasmic reticulum, independently of known retrieval motifs.. European Journal of Cell Biology Cytobiologie. 70:298-305. 1996
- Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases. Trends in Genetics. 12:170-170. 1996
- Dramatically Different Phenotypes in Mouse Models of Human Tay-Sachs and Sandhoff Diseases. Human Molecular Genetics. 5:1-14. 1996
- Molecular role of sulfated glycoprotein-1 (SGP-1/prosaposin) in Sertoli cells.. Histology and Histopathology. 10:1023-1034. 1995
- Ultrastructural and immunocytochemical study of skin fibroblasts from normal and sialidosis patients.. Cell and Tissue Research. 278:527-534. 1994
- PURIFICATION AND PARTIAL CHARACTERIZATION OF AN AUTOTOMY-PROMOTING FACTOR FROM THE SEA STAR PYCNOPODIA-HELIANTHOIDES. Biological Bulletin. 176:169-175. 1989
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conference paper
- SR-BI IN BONE MARROW DERIVED CELLS PROTECTS MICE FROM DIET INDUCED CORONARY ARTERY ATHEROSCLEROSIS AND MYOCARDIAL INFARCTION. Canadian Journal of Cardiology. S268-S268. 2013
- TRANSIENT NEONATAL HYPERPARATHYROIDISM: A PRESENTING FEATURE OF SIALIDOSIS TYPE II. Journal of Inherited Metabolic Disease. S101-S101. 2012
- Transcriptional induction of human lysosomal sialidase promoter as a potential strategy for the treatment of Tay Sachs disease. Molecular Genetics and Metabolism. S22-S22. 2011
- Immune cell phenotypes and cytokine response in a mouse model of sialidase deficiency. Molecular Genetics and Metabolism. S22-S22. 2010
- Impact of tumor necrosis factor-alpha deletion on astrolgliosis and microgliosis in the CNS of Sandhoff mice. Molecular Genetics and Metabolism. S22-S22. 2010
- Prevention of atherosclerosis in Apoe knockout mice using sialidase inhibitors: A new class of anti-atherogenic drugs. Atherosclerosis Supplements. 187-187. 2007
- Tissue-specific sialidase deficiency protects against atherosclerosis in experimental animals. Atherosclerosis Supplements. 488-488. 2006
- Long-term correction of sialidase deficiency in cells and mice by recombinant helper-dependent adenovirus-mediated gene transfer.. Genetics in Medicine. 248-248. 2004
- Molecular mechanisms of TNF alpha mediated hepatic leukocyte recruitment. FASEB Journal. A623-A623. 2004
- Differential roles of lysosomal and proteasomal degradation in the life-cycle of Cx43 in gap junctional intercellular communication-deficient and -competent breast tumor cells. Molecular Biology of the Cell Cell Regulation. 353A-353A. 2002
- Point mutations in the lysosomal sialidase gene cause tissue-specific sialidase deficiency in SM/J mice. Molecular Biology of the Cell Cell Regulation. 104A-104A. 2002
- HIGH EXPRESSION OF ALPHA-MANNOSIDASE IB IN PLACENTA AND INT HE NERVOUS-SYSTEM. Molecular Biology of the Cell Cell Regulation. 489-489. 1995
- SITE-SPECIFIC ANTIBODY RECOGNIZES THE LYSOSOMAL BUT NOT THE SECRETED FORM OF PROSAPOSIN SULFATED GLYCOPROTEIN-1 OF RAT SERTOLI CELLS. Molecular Biology of the Cell Cell Regulation. A12-A12. 1992