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Journal Abstract Search

148 related items for PubMed ID: 12770782

  • 21. Glycosphingolipid expression in human skeletal and heart muscle assessed by immunostaining thin-layer chromatography.
    Müthing J, Cacić M.
    Glycoconj J; 1997 Jan; 14(1):19-28. PubMed ID: 9076510
    [Abstract] [Full Text] [Related]

  • 22. Analysis of a splice-site mutation in the sap-precursor gene of a patient with metachromatic leukodystrophy.
    Henseler M, Klein A, Reber M, Vanier MT, Landrieu P, Sandhoff K.
    Am J Hum Genet; 1996 Jan; 58(1):65-74. PubMed ID: 8554069
    [Abstract] [Full Text] [Related]

  • 23. Sphingolipid activator proteins (SAPs) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL).
    Tyynelä J, Baumann M, Henseler M, Sandhoff K, Haltia M.
    Am J Med Genet; 1995 Jun 05; 57(2):294-7. PubMed ID: 7668348
    [Abstract] [Full Text] [Related]

  • 24. Neuronal Ganglioside and Glycosphingolipid (GSL) Metabolism and Disease : Cascades of Secondary Metabolic Errors Can Generate Complex Pathologies (in LSDs).
    Sandhoff R, Sandhoff K.
    Adv Neurobiol; 2023 Jun 05; 29():333-390. PubMed ID: 36255681
    [Abstract] [Full Text] [Related]

  • 25. A protein activator for the enzymic hydrolysis of GM2 ganglioside.
    Li SC, Hirabayashi Y, Li YT.
    J Biol Chem; 1981 Jun 25; 256(12):6234-40. PubMed ID: 7240201
    [No Abstract] [Full Text] [Related]

  • 26. Biosynthesis, processing, and targeting of sphingolipid activator protein (SAP )precursor in cultured human fibroblasts. Mannose 6-phosphate receptor-independent endocytosis of SAP precursor.
    Vielhaber G, Hurwitz R, Sandhoff K.
    J Biol Chem; 1996 Dec 13; 271(50):32438-46. PubMed ID: 8943309
    [Abstract] [Full Text] [Related]

  • 27. Interfacial regulation of acid ceramidase activity. Stimulation of ceramide degradation by lysosomal lipids and sphingolipid activator proteins.
    Linke T, Wilkening G, Sadeghlar F, Mozcall H, Bernardo K, Schuchman E, Sandhoff K.
    J Biol Chem; 2001 Feb 23; 276(8):5760-8. PubMed ID: 11104761
    [Abstract] [Full Text] [Related]

  • 28. Mutational analysis in a patient with a variant form of Gaucher disease caused by SAP-2 deficiency.
    Rafi MA, de Gala G, Zhang XL, Wenger DA.
    Somat Cell Mol Genet; 1993 Jan 23; 19(1):1-7. PubMed ID: 8460394
    [Abstract] [Full Text] [Related]

  • 29. Only sphingolipid activator protein B (SAP-B or saposin B) stimulates the degradation of globotriaosylceramide by recombinant human lysosomal alpha-galactosidase in a detergent-free liposomal system.
    Kase R, Bierfreund U, Klein A, Kolter T, Itoh K, Suzuki M, Hashimoto Y, Sandhoff K, Sakuraba H.
    FEBS Lett; 1996 Sep 09; 393(1):74-6. PubMed ID: 8804427
    [Abstract] [Full Text] [Related]

  • 30. Simultaneous deficiency of sphingolipid activator proteins 1 and 2 is caused by a mutation in the initiation codon of their common gene.
    Schnabel D, Schröder M, Fürst W, Klein A, Hurwitz R, Zenk T, Weber J, Harzer K, Paton BC, Poulos A.
    J Biol Chem; 1992 Feb 15; 267(5):3312-5. PubMed ID: 1371116
    [Abstract] [Full Text] [Related]

  • 31. Expression and specificity of human GM2 activator protein.
    Wu YY, Lockyer JM, Sugiyama E, Pavlova NV, Li YT, Li SC.
    J Biol Chem; 1994 Jun 10; 269(23):16276-83. PubMed ID: 8206933
    [Abstract] [Full Text] [Related]

  • 32. Characterization of a mutation in a family with saposin B deficiency: a glycosylation site defect.
    Kretz KA, Carson GS, Morimoto S, Kishimoto Y, Fluharty AL, O'Brien JS.
    Proc Natl Acad Sci U S A; 1990 Apr 10; 87(7):2541-4. PubMed ID: 2320574
    [Abstract] [Full Text] [Related]

  • 33. A novel mutation in the coding region of the prosaposin gene leads to a complete deficiency of prosaposin and saposins, and is associated with a complex sphingolipidosis dominated by lactosylceramide accumulation.
    Hulková H, Cervenková M, Ledvinová J, Tochácková M, Hrebícek M, Poupetová H, Befekadu A, Berná L, Paton BC, Harzer K, Böör A, Smíd F, Elleder M.
    Hum Mol Genet; 2001 Apr 15; 10(9):927-40. PubMed ID: 11309366
    [Abstract] [Full Text] [Related]

  • 34. Binding of GM1 ganglioside to a synthetic peptide derived from the lysosomal sphingolipid activator protein saposin B.
    Champagne MJ, Lamontagne S, Potier M.
    FEBS Lett; 1994 Aug 08; 349(3):439-41. PubMed ID: 8050611
    [Abstract] [Full Text] [Related]

  • 35. Binding of GM1-ganglioside to a synthetic peptide derived from the lysosomal sphingolipid-activator-protein saposin B.
    Champagne MJ, Lamontagne S, Potier M.
    FEBS Lett; 1994 Jun 27; 347(2-3):265-7. PubMed ID: 8034015
    [Abstract] [Full Text] [Related]

  • 36. Effect of saposins A and C on the enzymatic hydrolysis of liposomal glucosylceramide.
    Vaccaro AM, Tatti M, Ciaffoni F, Salvioli R, Barca A, Scerch C.
    J Biol Chem; 1997 Jul 04; 272(27):16862-7. PubMed ID: 9201993
    [Abstract] [Full Text] [Related]

  • 37. Isolation, characterization, and proteolysis of human prosaposin, the precursor of saposins (sphingolipid activator proteins).
    Hiraiwa M, O'Brien JS, Kishimoto Y, Galdzicka M, Fluharty AL, Ginns EI, Martin BM.
    Arch Biochem Biophys; 1993 Jul 04; 304(1):110-6. PubMed ID: 8323276
    [Abstract] [Full Text] [Related]

  • 38. Ceramide accumulation is associated with increased apoptotic cell death in cultured fibroblasts of sphingolipid activator protein-deficient mouse but not in fibroblasts of patients with Farber disease.
    Tohyama J, Oya Y, Ezoe T, Vanier MT, Nakayasu H, Fujita N, Suzuki K.
    J Inherit Metab Dis; 1999 Jun 04; 22(5):649-62. PubMed ID: 10399097
    [Abstract] [Full Text] [Related]

  • 39. Correction of sulfatide metabolism after transfer of prosaposin cDNA to cultured cells from a patient with SAP-1 deficiency.
    Rafi MA, Amini S, Zhang XL, Wenger DA.
    Am J Hum Genet; 1992 Jun 04; 50(6):1252-8. PubMed ID: 1350885
    [Abstract] [Full Text] [Related]

  • 40. Metabolic processing of gangliosides by normal and Salla human fibroblasts in culture. A study performed by administering radioactive GM3 ganglioside.
    Chigorno V, Tettamanti G, Sonnino S.
    J Biol Chem; 1996 Sep 06; 271(36):21738-44. PubMed ID: 8702969
    [Abstract] [Full Text] [Related]

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