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Annals of Medicine|April 30, 1998
The beta-hexosaminidase deficiency disorders: development of a clinical paradigm in the mouseC J Tifft, R L ProiaThe Journal of Biological Chemistry|May 5, 1989
Analysis of the glycosylation and phosphorylation of the lysosomal enzyme, beta-hexosaminidase B, by site-directed mutagenesisS Sonderfeld-Fresko, R L ProiaThe Journal of Biological Chemistry|September 15, 1988
Synthesis and assembly of a catalytically active lysosomal enzyme, beta-hexosaminidase B, in a cell-free systemS Sonderfeld-Fresko, R L ProiaAmerican Journal of Human Genetics|August 1, 1992
Identification and rapid detection of three Tay-Sachs mutations in the Moroccan Jewish populationL Drucker, R L Proia, R NavonBrain Pathology (Zurich, Switzerland)|February 11, 1998
Mouse models of human lysosomal diseasesK Suzuki, R L Proia, K SuzukiFEBS Letters|April 24, 1999
Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher miceT Doering, R L Proia, K SandhoffDNA and Cell Biology|February 1, 1996
Promoters for the human beta-hexosaminidase genes, HEXA and HEXBF Norflus, S Yamanaka, R L ProiaProceedings of the National Academy of Sciences of the United States of America|September 27, 2000
Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantationR Wada, C J Tifft, R L ProiaInfection and Immunity|December 1, 1979
Interaction of diphtheria toxin with phosphorylated moleculesR L Proia, D A Hart, L EidelsThe Journal of Biological Chemistry|March 10, 1984
Association of alpha- and beta-subunits during the biosynthesis of beta-hexosaminidase in cultured human fibroblastsR L Proia, A d'Azzo, E F NeufeldPageof 6