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Journal Abstract Search
633 related items for PubMed ID: 10333491
1. Stable expression of protective protein/cathepsin A-green fluorescent protein fusion genes in a fibroblastic cell line from a galactosialidosis patient. Model system for revealing the intracellular transport of normal and mutated lysosomal enzymes. Naganawa Y, Itoh K, Shimmoto M, Kamei S, Takiguchi K, Doi H, Sakuraba H. Biochem J; 1999 Jun 01; 340 ( Pt 2)(Pt 2):467-74. PubMed ID: 10333491 [Abstract] [Full Text] [Related]
2. Expression of lysosomal protective protein/cathepsin A in a stably transformed human neuroblastoma cell line during bi-directional differentiation into neuronal and Schwannian cells. Itoh K, Satoh Y, Kadota Y, Oheda Y, Kuwahara J, Shimmoto M, Sakuraba H. Neurochem Int; 2004 May 01; 44(6):447-57. PubMed ID: 14687610 [Abstract] [Full Text] [Related]
3. Protective protein/cathepsin A loss in cultured cells derived from an early-infantile form of galactosialidosis patients homozygous for the A1184-G transition (Y395C mutation). Itoh K, Shimmoto M, Utsumi K, Mizoguchi N, Miharu N, Ohama K, Sakuraba H. Biochem Biophys Res Commun; 1998 Jun 09; 247(1):12-7. PubMed ID: 9636645 [Abstract] [Full Text] [Related]
4. Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A. van der Spoel A, Bonten E, d'Azzo A. EMBO J; 1998 Mar 16; 17(6):1588-97. PubMed ID: 9501080 [Abstract] [Full Text] [Related]
5. Observing proteases in living cells. Moin K, Demchik L, Mai J, Duessing J, Peters C, Sloane BF. Adv Exp Med Biol; 2000 Mar 16; 477():391-401. PubMed ID: 10849765 [Abstract] [Full Text] [Related]
10. The atomic model of the human protective protein/cathepsin A suggests a structural basis for galactosialidosis. Rudenko G, Bonten E, Hol WG, d'Azzo A. Proc Natl Acad Sci U S A; 1998 Jan 20; 95(2):621-5. PubMed ID: 9435242 [Abstract] [Full Text] [Related]
13. Functional amelioration of murine galactosialidosis by genetically modified bone marrow hematopoietic progenitor cells. Leimig T, Mann L, Martin Mdel P, Bonten E, Persons D, Knowles J, Allay JA, Cunningham J, Nienhuis AW, Smeyne R, d'Azzo A. Blood; 2002 May 01; 99(9):3169-78. PubMed ID: 11964280 [Abstract] [Full Text] [Related]
14. Molecular and biochemical analysis of protective protein/cathepsin A mutations: correlation with clinical severity in galactosialidosis. Zhou XY, van der Spoel A, Rottier R, Hale G, Willemsen R, Berry GT, Strisciuglio P, Morrone A, Zammarchi E, Andria G, d'Azzo A. Hum Mol Genet; 1996 Dec 01; 5(12):1977-87. PubMed ID: 8968752 [Abstract] [Full Text] [Related]
16. Rab7: a key to lysosome biogenesis. Bucci C, Thomsen P, Nicoziani P, McCarthy J, van Deurs B. Mol Biol Cell; 2000 Feb 01; 11(2):467-80. PubMed ID: 10679007 [Abstract] [Full Text] [Related]
17. New mutations in the PPBG gene lead to loss of PPCA protein which affects the level of the beta-galactosidase/neuraminidase complex and the EBP-receptor. Malvagia S, Morrone A, Caciotti A, Bardelli T, d'Azzo A, Ancora G, Zammarchi E, Donati MA. Mol Genet Metab; 2004 May 01; 82(1):48-55. PubMed ID: 15110321 [Abstract] [Full Text] [Related]
19. Biosynthesis and intracellular transport of alpha-glucosidase and cathepsin D in normal and mutant human fibroblasts. Oude Elferink RP, Van Doorn-Van Wakeren J, Strijland A, Reuser AJ, Tager JM. Eur J Biochem; 1985 Nov 15; 153(1):55-63. PubMed ID: 3905406 [Abstract] [Full Text] [Related]