Proia RL - Search Results

1

Kolter T, Proia RL, Sandhoff K. Kolter T, et al. Among authors: proia rl. J Biol Chem. 2002 Jul 19;277(29):25859-62. doi: 10.1074/jbc.R200001200. Epub 2002 May 13. J Biol Chem. 2002. PMID: 12011101 Free article. Review. No abstract available.

2

Physiological substrates for human lysosomal beta -hexosaminidase S.

Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K. Hepbildikler ST, et al. Among authors: proia rl. J Biol Chem. 2002 Jan 25;277(4):2562-72. doi: 10.1074/jbc.M105457200. Epub 2001 Nov 13. J Biol Chem. 2002. PMID: 11707436 Free article.

3

Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures.

Kawai H, Allende ML, Wada R, Kono M, Sango K, Deng C, Miyakawa T, Crawley JN, Werth N, Bierfreund U, Sandhoff K, Proia RL. Kawai H, et al. Among authors: proia rl. J Biol Chem. 2001 Mar 9;276(10):6885-8. doi: 10.1074/jbc.C000847200. Epub 2000 Dec 27. J Biol Chem. 2001. PMID: 11133999 Free article.

4

Identification of the protein receptor binding site of botulinum neurotoxins B and G proves the double-receptor concept.

Rummel A, Eichner T, Weil T, Karnath T, Gutcaits A, Mahrhold S, Sandhoff K, Proia RL, Acharya KR, Bigalke H, Binz T. Rummel A, et al. Among authors: proia rl. Proc Natl Acad Sci U S A. 2007 Jan 2;104(1):359-64. doi: 10.1073/pnas.0609713104. Epub 2006 Dec 21. Proc Natl Acad Sci U S A. 2007. PMID: 17185412 Free PMC article.

5

Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher mice.

Doering T, Proia RL, Sandhoff K. Doering T, et al. Among authors: proia rl. FEBS Lett. 1999 Mar 26;447(2-3):167-70. doi: 10.1016/s0014-5793(99)00274-4. FEBS Lett. 1999. PMID: 10214939 Free article.

6

Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions.

Yamashita T, Wu YP, Sandhoff R, Werth N, Mizukami H, Ellis JM, Dupree JL, Geyer R, Sandhoff K, Proia RL. Yamashita T, et al. Among authors: proia rl. Proc Natl Acad Sci U S A. 2005 Feb 22;102(8):2725-30. doi: 10.1073/pnas.0407785102. Epub 2005 Feb 14. Proc Natl Acad Sci U S A. 2005. PMID: 15710896 Free PMC article.

7

Identification of domains in human beta-hexosaminidase that determine substrate specificity.

Pennybacker M, Liessem B, Moczall H, Tifft CJ, Sandhoff K, Proia RL. Pennybacker M, et al. Among authors: proia rl. J Biol Chem. 1996 Jul 19;271(29):17377-82. doi: 10.1074/jbc.271.29.17377. J Biol Chem. 1996. PMID: 8663217 Free article.

8

Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency.

Potratz A, Hüttler S, Bierfreund U, Proia RL, Suzuki K, Sandhoff K. Potratz A, et al. Among authors: proia rl. Biochim Biophys Acta. 2000 Nov 15;1502(3):391-7. doi: 10.1016/s0925-4439(00)00063-6. Biochim Biophys Acta. 2000. PMID: 11068181 Free article.

9

Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism.

Sango K, Yamanaka S, Hoffmann A, Okuda Y, Grinberg A, Westphal H, McDonald MP, Crawley JN, Sandhoff K, Suzuki K, Proia RL. Sango K, et al. Among authors: proia rl. Nat Genet. 1995 Oct;11(2):170-6. doi: 10.1038/ng1095-170. Nat Genet. 1995. PMID: 7550345

10

Evidence for the involvement of Glu-355 in the catalytic action of human beta-hexosaminidase B.

Pennybacker M, Schuette CG, Liessem B, Hepbildikler ST, Kopetka JA, Ellis MR, Myerowitz R, Sandhoff K, Proia RL. Pennybacker M, et al. Among authors: proia rl. J Biol Chem. 1997 Mar 21;272(12):8002-6. doi: 10.1074/jbc.272.12.8002. J Biol Chem. 1997. PMID: 9065471 Free article.

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