Plotz PH - Search Results

1

Role of autophagy in the pathogenesis of Pompe disease.

Raben N, Roberts A, Plotz PH. Raben N, et al. Among authors: plotz ph. Acta Myol. 2007 Jul;26(1):45-8. Acta Myol. 2007. PMID: 17915569 Free PMC article. Review.

2

Acid alpha-glucosidase deficiency (glycogenosis type II, Pompe disease).

Raben N, Plotz P, Byrne BJ. Raben N, et al. Curr Mol Med. 2002 Mar;2(2):145-66. doi: 10.2174/1566524024605789. Curr Mol Med. 2002. PMID: 11949932 Review.

3

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. Raben N, et al. Hum Mol Genet. 2008 Dec 15;17(24):3897-908. doi: 10.1093/hmg/ddn292. Epub 2008 Sep 9. Hum Mol Genet. 2008. PMID: 18782848 Free PMC article.

4

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. Raben N, et al. Autophagy. 2009 Jan;5(1):111-3. doi: 10.4161/auto.5.1.7293. Epub 2009 Jan 30. Autophagy. 2009. PMID: 19001870 Free PMC article.

5

Impaired organization and function of myofilaments in single muscle fibers from a mouse model of Pompe disease.

Xu S, Galperin M, Melvin G, Horowits R, Raben N, Plotz P, Yu L. Xu S, et al. J Appl Physiol (1985). 2010 May;108(5):1383-8. doi: 10.1152/japplphysiol.01253.2009. Epub 2010 Mar 11. J Appl Physiol (1985). 2010. PMID: 20223998 Free PMC article.

6

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 PH. Raben N, et al. Among authors: plotz ph. Mol Ther. 2002 Nov;6(5):601-8. Mol Ther. 2002. PMID: 12409258 Free article.

7

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 AL, de Jong D, Thurberg BL, Mattaliano RJ, Meikle P, Hopwood JJ, Nagashima K, Nagaraju K, Plotz PH. Raben N, et al. Among authors: plotz ph. Mol Ther. 2005 Jan;11(1):48-56. doi: 10.1016/j.ymthe.2004.09.017. Mol Ther. 2005. PMID: 15585405 Free article.

8

Dysfunction of endocytic and autophagic pathways in a lysosomal storage disease.

Fukuda T, Ewan L, Bauer M, Mattaliano RJ, Zaal K, Ralston E, Plotz PH, Raben N. Fukuda T, et al. Among authors: plotz ph. Ann Neurol. 2006 Apr;59(4):700-8. doi: 10.1002/ana.20807. Ann Neurol. 2006. PMID: 16532490

9

Autophagy and lysosomes in Pompe disease.

Fukuda T, Roberts A, Ahearn M, Zaal K, Ralston E, Plotz PH, Raben N. Fukuda T, et al. Among authors: plotz ph. Autophagy. 2006 Oct-Dec;2(4):318-20. doi: 10.4161/auto.2984. Epub 2006 Oct 5. Autophagy. 2006. PMID: 16874053

10

Autophagy and mistargeting of therapeutic enzyme in skeletal muscle in Pompe disease.

Fukuda T, Ahearn M, Roberts A, Mattaliano RJ, Zaal K, Ralston E, Plotz PH, Raben N. Fukuda T, et al. Among authors: plotz ph. Mol Ther. 2006 Dec;14(6):831-9. doi: 10.1016/j.ymthe.2006.08.009. Epub 2006 Sep 27. Mol Ther. 2006. PMID: 17008131 Free PMC article.

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