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32 results on '"Dwyer, Nancy K."'

3. Constitutive Achlorhydria in Mucolipidosis Type IV

Author

Schiffmann, Raphael, Dwyer, Nancy K., Lubensky, Irina A., Tsokos, Maria, Sutliff, Vincent E., Latimer, John S., Frei, Karen P., Brady, Roscoe O., Barton, Norman W., Blanchette-Mackie, E. Joan, and Goldin, Ehud

Published
1998

4. Niemann-Pick C1 Disease Gene: Homology to Mediators of Cholesterol Homeostasis

Author

Carstea, Eugene D., Morris, Jill A., Coleman, Katherine G., Loftus, Stacie K., Zhang, Dana, Cummings, Christiano, Gu, Jessie, Rosenfeld, Melissa A., Pavan, William J., Krizman, David B., Nagle, James, Polymeropoulos, Mihail H., Sturley, Stephen L., Ioannou, Yiannis A., Higgins, Maureen E., Comly, Marcella, Cooney, Adele, Brown, Anthony, Kaneski, Christine R., Blanchette-Mackie, E. Joan, Dwyer, Nancy K., Neufeld, Edward B., Chang, Ta-Yuan, Liscum, Laura, Strauss, Jerome F., Ohno, Kousaku, Zeigler, Marsha, Carmi, Rivka, Sokol, Jacob, Markie, David, O'Neill, Raymond R., van Diggelen, O. P., Elleder, Milan, Patterson, Marc C., Brady, Roscoe O., Vanier, Marie T., Pentchev, Peter G., and Tagle, Danilo A.

Published
1997

5. Conceptualizing Ratios with Look-Alike Polygons.

Author

Dwyer, Nancy K., Causey-Lee, Betty J., and Irby, Nekeya M.

Abstract

Presents an activity using similar rectangles to help children conceptualize ratio relationships in several different ways. Develops proportional reasoning without introducing the process of cross- multiplication. (YDS)

Published
2003

11. Transduction of rat pancreatic islets with pseudotyped adeno-associated virus vectors

Author

Blanchette-Mackie E Joan, Chiorini John A, McAlister Victor J, Schmidt Michael, Pechhold Klaus, Liu Eric, Pack Stephanie, Jou William, Dwyer Nancy K, Gavrilova Oksana, Craig Anthony T, Harlan David M, and Owens Roland A

Subjects
Infectious and parasitic diseases, RC109-216
Abstract

Abstract Background Pancreatic islet transplantation is a promising treatment for type I diabetes mellitus, but current immunosuppressive strategies do not consistently provide long-term survival of transplanted islets. We are therefore investigating the use of adeno-associated viruses (AAVs) as gene therapy vectors to transduce rat islets with immunosuppressive genes prior to transplantation into diabetic mice. Results We compared the transduction efficiency of AAV2 vectors with an AAV2 capsid (AAV2/2) to AAV2 vectors pseudotyped with AAV5 (AAV2/5), AAV8 (AAV2/8) or bovine adeno-associated virus (BAAV) capsids, or an AAV2 capsid with an insertion of the low density lipoprotein receptor ligand from apolipoprotein E (AAV2apoE), on cultured islets, in the presence of helper adenovirus infection to speed expression of a GFP transgene. Confocal microscopy and flow cytometry were used. The AAV2/5 vector was superior to AAV2/2 and AAV2/8 in rat islets. Flow cytometry indicated AAV2/5-mediated gene expression in approximately 9% of rat islet cells and almost 12% of insulin-positive cells. The AAV2/8 vector had a higher dependence on the helper virus multiplicity of infection than the AAV 2/5 vector. In addition, the BAAV and AAV2apoE vectors were superior to AAV2/2 for transducing rat islets. Rat islets (300 per mouse) transduced with an AAV2/5 vector harboring the immunosuppressive transgene, tgfβ1, retain the ability to correct hyperglycemia when transplanted into immune-deficient diabetic mice. Conclusion AAV2/5 vectors may therefore be useful for pre-treating donor islets prior to transplantation.

Published
2009
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13. Cultured Skin Fibroblasts Derived from Patients with Mucolipidosis 4 Are Auto-Fluorescent

Author

Goldin, Ehud, Blanchette-Mackie, E. Joan, Joan Blanchette Mackie, E., Dwyer, Nancy K., Pentchev, Peter G., and Brady, Roscoe O.

Abstract

Mucolipidosis 4 (ML4) is an autosomal recessive disorder with both lipid and mucopolysaccharide storage. The disease is characterized by severe visual impairment and psychomotor retardation. In our effort to find a phenotypic marker for ML4 fibroblasts, living cells were stained with fluorescent compounds. The staining pattern in cells was complicated by autofluorescence. A careful study revealed that auto-fluorescence by itself was a sufficient marker for viable ML4 fibroblasts. ML4 cells in cultures obtained from four unrelated patients contain auto-fluorescent material. Auto-fluorescence was noted over a wide range of excitation wavelengths from approximately 365 to approximately 546 nm. The most intense fluorescence was observed in the lower wave-length range. Cultured fibroblasts from normal individuals or obligate ML4 heterozygotes did not fluoresce under adequately controlled culture conditions. High passage number of inadequate feeding caused a small proportion of fibroblasts obtained from normal individuals to auto-fluoresce. The auto-fluorescent material co-localized with phase-dense inclusion bodies, shown to be lysosomes by staining with LAMP-ab. These findings imply that fluorescence may relate to the specific compound(s) stored in the lysosomes. In a comparative study, neuronal ceroid lipofuscinosis fibroblasts were also fluorescent. Fibroblasts from other diseases such as Gaucher disease and glycogenosis type 2 did not show any fluorescence. These findings are currently used in our functional cloning strategy for determining the gene involved in ML4.

Published
1995

14. Transduction of rat pancreatic islets with pseudotyped adeno-associated virus vectors

Author

Craig, Anthony T, primary, Gavrilova, Oksana, additional, Dwyer, Nancy K, additional, Jou, William, additional, Pack, Stephanie, additional, Liu, Eric, additional, Pechhold, Klaus, additional, Schmidt, Michael, additional, McAlister, Victor J, additional, Chiorini, John A, additional, Blanchette-Mackie, E Joan, additional, Harlan, David M, additional, and Owens, Roland A, additional

Published
2009
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17. Cellular Localization and Trafficking of the Human ABCA1 Transporter

Author

Neufeld, Edward B., primary, Remaley, Alan T., additional, Demosky, Stephen J., additional, Stonik, John A., additional, Cooney, Adele M., additional, Comly, Marcy, additional, Dwyer, Nancy K., additional, Zhang, Mei, additional, Blanchette-Mackie, Joan, additional, Santamarina-Fojo, Silvia, additional, and Brewer, H. Bryan, additional

Published
2001
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18. Sterol-modulated Glycolipid Sorting Occurs in Niemann-Pick C1 Late Endosomes

Author

Zhang, Mei, primary, Dwyer, Nancy K., additional, Neufeld, Edward B., additional, Love, Dona C., additional, Cooney, Adele, additional, Comly, Marcy, additional, Patel, Shutish, additional, Watari, Hidemichi, additional, Strauss, Jerome F., additional, Pentchev, Peter G., additional, Hanover, John A., additional, and Blanchette-Mackie, E. Joan, additional

Published
2001
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22. The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo

Author

Neufeld, Edward B., primary, Wastney, Meryl, additional, Patel, Shutish, additional, Suresh, Sundar, additional, Cooney, Adele M., additional, Dwyer, Nancy K., additional, Roff, Calvin F., additional, Ohno, Kousaku, additional, Morris, Jill A., additional, Carstea, Eugene D., additional, Incardona, John P., additional, Strauss, Jerome F., additional, Vanier, Marie T., additional, Patterson, Marc C., additional, Brady, Roscoe O., additional, Pentchev, Peter G., additional, and Blanchette-Mackie, E. Joan, additional

Published
1999
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29. Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease.

Author

Mei Zhang, Dwyer, Nancy K., Love, Dona C., Cooney, Adele, Comly, Marcy, Neufeld, Edward, Pentchev, Peter G., Blanchette-Mackie, E. Joan, and Hanover, John A.

Subjects
*NEURODEGENERATION, *CELLULAR pathology
Abstract

Studies the underlying cellular pathology in neurodegenerative Nieman-Pick type C1 (NPC1) disease. Biosynthesis and trafficking of NCP1; Exchange of contents between late endosomes and lysosomes; Results indicating that NPC1 disease results from disordered endocytic tubulovesicular exchange.

Published
2001
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