Nina Raben
Arthritis and Rheumatism Branch
NIAMS
NIH, Bethesda
MD 20892-1820
USA
Name/email consistency: high
- When more is less: excess and deficiency of autophagy coexist in skeletal muscle in Pompe disease. Raben, N., Baum, R., Schreiner, C., Takikita, S., Mizushima, N., Ralston, E., Plotz, P. Autophagy (2009)
- Suppression of autophagy in skeletal muscle uncovers the accumulation of ubiquitinated proteins and their potential role in muscle damage in Pompe disease. Raben, N., Hill, V., Shea, L., Takikita, S., Baum, R., Mizushima, N., Ralston, E., Plotz, P. Hum. Mol. Genet. (2008)
- Deconstructing Pompe disease by analyzing single muscle fibers: to see a world in a grain of sand... Raben, N., Takikita, S., Pittis, M.G., Bembi, B., Marie, S.K., Roberts, A., Page, L., Kishnani, P.S., Schoser, B.G., Chien, Y.H., Ralston, E., Nagaraju, K., Plotz, P.H. Autophagy (2007)
- Replacing acid alpha-glucosidase in Pompe disease: recombinant and transgenic enzymes are equipotent, but neither completely clears glycogen from type II muscle fibers. Raben, N., Fukuda, T., Gilbert, A.L., de Jong, D., Thurberg, B.L., Mattaliano, R.J., Meikle, P., Hopwood, J.J., Nagashima, K., Nagaraju, K., Plotz, P.H. Mol. Ther. (2005)
- Induction of tolerance to a recombinant human enzyme, acid alpha-glucosidase, in enzyme deficient knockout mice. Raben, N., Nagaraju, K., Lee, A., Lu, N., Rivera, Y., Jatkar, T., Hopwood, J.J., Plotz, P.H. Transgenic Res. (2003)
- Enzyme replacement therapy in the mouse model of Pompe disease. Raben, N., Danon, M., Gilbert, A.L., Dwivedi, S., Collins, B., Thurberg, B.L., Mattaliano, R.J., Nagaraju, K., Plotz, P.H. Mol. Genet. Metab. (2003)
- Acid alpha-glucosidase deficiency (glycogenosis type II, Pompe disease). Raben, N., Plotz, P., Byrne, B.J. Curr. Mol. Med. (2002)
- Glycogen stored in skeletal but not in cardiac muscle in acid alpha-glucosidase mutant (Pompe) mice is highly resistant to transgene-encoded human enzyme. Raben, N., Jatkar, T., Lee, A., Lu, N., Dwivedi, S., Nagaraju, K., Plotz, P.H. Mol. Ther. (2002)
- Surprises of genetic engineering: a possible model of polyglucosan body disease. Raben, N., Danon, M., Lu, N., Lee, E., Shliselfeld, L., Skurat, A.V., Roach, P.J., Lawrence, J.C., Musumeci, O., Shanske, S., DiMauro, S., Plotz, P. Neurology (2001)
- Conditional tissue-specific expression of the acid alpha-glucosidase (GAA) gene in the GAA knockout mice: implications for therapy. Raben, N., Lu, N., Nagaraju, K., Rivera, Y., Lee, A., Yan, B., Byrne, B., Meikle, P.J., Umapathysivam, K., Hopwood, J.J., Plotz, P.H. Hum. Mol. Genet. (2001)
- Modulation of disease severity in mice with targeted disruption of the acid alpha-glucosidase gene. Raben, N., Nagaraju, K., Lee, E., Plotz, P. Neuromuscul. Disord. (2000)
- Targeted disruption of the acid alpha-glucosidase gene in mice causes an illness with critical features of both infantile and adult human glycogen storage disease type II. Raben, N., Nagaraju, K., Lee, E., Kessler, P., Byrne, B., Lee, L., LaMarca, M., King, C., Ward, J., Sauer, B., Plotz, P. J. Biol. Chem. (1998)