selected scholarly activity
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chapters
- Sialidosis. V3-V3-115. 2010
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conferences
- Loss-of-function PCSK9 Mutants Evade the Unfolded Protein Response Sensor, GRP78, and Fail to Induce Endoplasmic Reticulum Stress when Retained. Atherosclerosis Plus. 139-139. 2018
- SR-BI in Bone Marrow-Derived Cells Protects Mice From Diet-Induced Coronary Artery Atherosclerosis and Myocardial Infarction. Arteriosclerosis, Thrombosis and Vascular Biology. 2014
- 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
- 67. Immune cell phenotypes and cytokine response in a mouse model of sialidase deficiency. Molecular Genetics and Metabolism. S22-S22. 2010
- 68. Impact of tumor necrosis factor-alpha deletion on astrolgliosis and microgliosis in the CNS of Sandhoff mice. Molecular Genetics and Metabolism. S22-S22. 2010
- Characterization of the hepatic endothelial glycocalyx of sialidase deficient mice in response to TNFα. The FASEB Journal. 2008
- PO22-699 PREVENTION OF ATHEROSCLEROSIS IN APOE KNOCKOUT MICE USING SIALIDASE INHIBITORS: A NEW CLASS OF ANTI-ATHEROGENIC DRUGS. Atherosclerosis Plus. 187-187. 2007
- Th-W60:6 Tissue-specific sialidase deficiency protects against atherosclerosis in experimental animals. Atherosclerosis Plus. 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. The 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. 353A-353A. 2002
- Point mutations in the lysosomal sialidase gene cause tissue-specific sialidase deficiency in SM/J mice. Molecular Biology of the Cell. 104A-104A. 2002
- HIGH EXPRESSION OF ALPHA-MANNOSIDASE IB IN PLACENTA AND INT HE NERVOUS-SYSTEM. Molecular Biology of the Cell. 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. A12-A12. 1992
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journal articles
- 4-Phenylbutyric acid mitigates ER stress-induced neurodegeneration in the spinal cords of a GM2 gangliosidosis mouse model.. Human Molecular Genetics. 34:32-46. 2025
- Scavenger receptor class B type I knockout mice develop extensive diet-induced coronary artery atherosclerosis in an age-dependent manner.. PLoS ONE. 20:e0318118. 2025
- GDF10 is a negative regulator of vascular calcification.. Journal of Biological Chemistry. 300:107805. 2024
- A new method for the purification of bioviable NEU1 sialidase for enzyme replacement therapy for sialidosis. Molecular Genetics and Metabolism. 141:107892-107892. 2024
- Impact of ASGR1 on the hepatic expression and localization of NEU1. Molecular Genetics and Metabolism. 141:107757-107757. 2024
- Nuclear localization of the NEU4 isoforms in human fibroblasts and HepG2 cell line. Molecular Genetics and Metabolism. 141:107995-107995. 2024
- Restoration of the ER stress response protein TDAG51 in hepatocytes mitigates NAFLD in mice. Journal of Biological Chemistry. 300:105655-105655. 2024
- Neuraminidase 4 (NEU4): new biological and physiological player. Glycobiology. 33:182-187. 2023
- Differential expression and localization of NEU1 and NEU4 in the CNS of mouse models of GM2 gangliosidoses. Molecular Genetics and Metabolism. 138:107261-107261. 2023
- Inhibitory Antibodies against PCSK9 Reduce Surface CD36 and Mitigate Diet-Induced Renal Lipotoxicity. Kidney360. 3:1394-1410. 2022
- Caffeine blocks SREBP2-induced hepatic PCSK9 expression to enhance LDLR-mediated cholesterol clearance. Nature Communications. 13:770. 2022
- Endoplasmic reticulum stress derives neurodegeneration in the spinal cord of Sandhoff disease mice. Molecular Genetics and Metabolism. 135:S127-S127. 2022
- Extensive diet-induced atherosclerosis in scavenger receptor class B type 1-deficient mice is associated with substantial leukocytosis and elevated vascular cell adhesion molecule-1 expression in coronary artery endothelium. Frontiers in Physiology. 13:1023397. 2022
- TDAG51 (T-Cell Death-Associated Gene 51) Is a Key Modulator of Vascular Calcification and Osteogenic Transdifferentiation of Arterial Smooth Muscle Cells. Arteriosclerosis, Thrombosis and Vascular Biology. 40:1664-1679. 2020
- Pcsk9 knockout exacerbates diet-induced non-alcoholic steatohepatitis, fibrosis and liver injury in mice. JHEP Reports. 1:418-429. 2019
- Dysfunctional autophagy impairs muscle regeneration in lysosomal diseases. Molecular Genetics and Metabolism. 126:S114-S114. 2019
- Sialidase down-regulation reduces non-HDL cholesterol, inhibits leukocyte transmigration, and attenuates atherosclerosis in ApoE knockout mice. Journal of Biological Chemistry. 293:14689-14706. 2018
- Loss-of-function PCSK9 mutants evade the unfolded protein response sensor GRP78 and fail to induce endoplasmic reticulum stress when retained. Journal of Biological Chemistry. 293:7329-7343. 2018
- Loss‐of‐function PCSK9 mutants evade the unfolded protein response sensor, GRP78, and fail to induce endoplasmic reticulum stress when retained. The FASEB Journal. 32:793.7-793.7. 2018
- 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:ddw422-ddw422. 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:e003945. 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
- Abstract 669: SR-BI in Bone Marrow--Derived Cells Protects Mice From Diet-Induced Coronary Artery Atherosclerosis and Myocardial Infarction. Arteriosclerosis, Thrombosis and Vascular Biology. 34. 2014
- Deletion of tumor necrosis factor-α ameliorates neurodegeneration in Sandhoff disease mice. Human Molecular Genetics. 22:3960-3975. 2013
- 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-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
- 69. The therapeutic potential of small pharmacological molecules in the treatment of Sialidosis. Molecular Genetics and Metabolism. 99:S22-S22. 2010
- 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
- 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
- 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
- Overexpression of MyoD-inducible lysosomal sialidase (neu1) inhibits myogenesis in C2C12 cells. Experimental Cell Research. 311:157-166. 2005
- Functional rescue of vitamin C synthesis deficiency in human cells using adenoviral-based expression of murine l-gulono-γ-lactone oxidase. Genomics. 83:482-492. 2004
- 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. 1001:37-50. 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
- Characterization of the mouse lysosomal sialidase promoter. Gene. 319:177-187. 2003
- Erratum. Molecular Biology and Evolution (MBE). 20:1946-1946. 2003
- Unmelanized Plumage Patterns in Old World Leaf Warblers Do Not Correspond to Sequence Variation at the Melanocortin-1 Receptor Locus (MC1R). Molecular Biology and Evolution (MBE). 20:1675-1681. 2003
- Lysosomal and Proteasomal Degradation Play Distinct Roles in the Life Cycle of Cx43 in Gap Junctional Intercellular Communication-deficient and -competent Breast Tumor Cells. Journal of Biological Chemistry. 278:30005-30014. 2003
- 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
- α-Mannosidases involved in N-glycan processing show cell specificity and distinct subcompartmentalization within the Golgi apparatus of cells in the testis and epididymis. European Journal of Cell Biology. 78:441-452. 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
- Low Density Lipoprotein Receptor‐Related Protein‐1 Expression in the Testis: Regulated Expression in Sertoli Cells. Journal of Andrology. 18:400-410. 1997
- Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis. Nature Genetics. 15:316-320. 1997
- Low Density Lipoprotein Receptor-Related Protein-2 Expression in Efferent Duct and Epididymal Epithelia: Evidence in Rats for its in Vivo Role in Endocytosis of Apolipoprotein J/Clusterin1. Biology of Reproduction. 55:676-683. 1996
- The Saccharomyces cerevisiae processing alpha 1,2-mannosidase is localized in the endoplasmic reticulum, independently of known retrieval motifs.. European Journal of Cell Biology. 70:298-305. 1996
- Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases. Trends in Genetics. 12:170-170. 1996
- Expression and tissue distribution of rat sulfated glycoprotein-1 (prosaposin).. Journal of Histochemistry and Cytochemistry. 44:327-337. 1996
- Trafficking of sulfated glycoprotein-1 (prosaposin) to lysosomes or to the extracellular space in rat Sertoli cells. Cell and Tissue Research. 283:385-394. 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
- Targeting of endogenous sulfated glycoprotein‐1 and ‐2 to lysosomes within nonciliated cells of the efferent ducts during postnatal development of the rat. Molecular Reproduction and Development. 41:287-299. 1995
- Differential expression of cathepsins B and D in testis and epididymis of adult rats.. Journal of Histochemistry and Cytochemistry. 43:545-557. 1995
- Hormonal regulation of sulfated glycoprotein‐1 synthesis by nonciliated cells of the efferent ducts of adult rats. Molecular Reproduction and Development. 40:69-83. 1995
- Role of sulfated glycoprotein‐1 (SGP‐1) in the disposal of residual bodies by sertoli cells of the rat. Molecular Reproduction and Development. 40:91-102. 1995
- Sulfated glycoprotein‐2 synthesized by nonciliated cells of the efferent ducts is targeted to the lysosomal compartment. Microscopy Research and Technique. 29:468-480. 1994
- Ultrastructural and immunocytochemical study of skin fibroblasts from normal and sialidosis patients. Cell and Tissue Research. 278:527-534. 1994
- Suppression of Spermatogenesis by Low‐Level Glycerol Treatment. Journal of Andrology. 15:234-243. 1994
- Nonciliated cells of the rat efferent ducts endocytose testicular sulfated glycoprotein‐1 (SGP‐1) and synthesize SGP‐1 derived saposins. Anatomical Record. 235:411-424. 1993
- Purification and Partial Characterization of an Autotomy-Promoting Factor from the Sea StarPycnopodia helianthoides. Biological Bulletin. 176:169-175. 1989