These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
297 related articles for article (PubMed ID: 8370580)
1. Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs. Multiple glycolipid elevations (including lactosylceramidosis), partial enzyme deficiencies and ultrastructure of the skin in this generalized sphingolipid storage disease. Bradová V; Smíd F; Ulrich-Bott B; Roggendorf W; Paton BC; Harzer K Hum Genet; 1993 Sep; 92(2):143-52. PubMed ID: 8370580 [TBL] [Abstract][Full Text] [Related]
2. 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; 10(9):927-40. PubMed ID: 11309366 [TBL] [Abstract][Full Text] [Related]
3. Metabolism of GM1 ganglioside in cultured skin fibroblasts: anomalies in gangliosidoses, sialidoses, and sphingolipid activator protein (SAP, saposin) 1 and prosaposin deficient disorders. Schmid B; Paton BC; Sandhoff K; Harzer K Hum Genet; 1992 Jul; 89(5):513-8. PubMed ID: 1634229 [TBL] [Abstract][Full Text] [Related]
4. Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase. Paton BC; Schmid B; Kustermann-Kuhn B; Poulos A; Harzer K Biochem J; 1992 Jul; 285 ( Pt 2)(Pt 2):481-8. PubMed ID: 1637339 [TBL] [Abstract][Full Text] [Related]
5. Sphingolipid activator protein deficiency in a 16-week-old atypical Gaucher disease patient and his fetal sibling: biochemical signs of combined sphingolipidoses. Harzer K; Paton BC; Poulos A; Kustermann-Kuhn B; Roggendorf W; Grisar T; Popp M Eur J Pediatr; 1989 Oct; 149(1):31-9. PubMed ID: 2514102 [TBL] [Abstract][Full Text] [Related]
6. Neuronopathic juvenile glucosylceramidosis due to sap-C deficiency: clinical course, neuropathology and brain lipid composition in this Gaucher disease variant. Pàmpols T; Pineda M; Girós ML; Ferrer I; Cusi V; Chabás A; Sanmarti FX; Vanier MT; Christomanou H Acta Neuropathol; 1999 Jan; 97(1):91-7. PubMed ID: 9930900 [TBL] [Abstract][Full Text] [Related]
7. Model SV40-transformed fibroblast lines for metabolic studies of human prosaposin and acid ceramidase deficiencies. Chatelut M; Harzer K; Christomanou H; Feunteun J; Pieraggi MT; Paton BC; Kishimoto Y; O'Brien JS; Basile JP; Thiers JC; Salvayre R; Levade T Clin Chim Acta; 1997 Jun; 262(1-2):61-76. PubMed ID: 9204210 [TBL] [Abstract][Full Text] [Related]
8. Accumulation of sphingolipids in SAP-precursor (prosaposin)-deficient fibroblasts occurs as intralysosomal membrane structures and can be completely reversed by treatment with human SAP-precursor. Burkhardt JK; Hüttler S; Klein A; Möbius W; Habermann A; Griffiths G; Sandhoff K Eur J Cell Biol; 1997 May; 73(1):10-8. PubMed ID: 9174667 [TBL] [Abstract][Full Text] [Related]
9. Physiological relevance of sphingolipid activator proteins in cultured human fibroblasts. Sadeghlar F; Remmel N; Breiden B; Klingenstein R; Schwarzmann G; Sandhoff K Biochimie; 2003; 85(3-4):439-48. PubMed ID: 12770782 [TBL] [Abstract][Full Text] [Related]
10. Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking. Sun Y; Witte DP; Zamzow M; Ran H; Quinn B; Matsuda J; Grabowski GA Hum Mol Genet; 2007 Apr; 16(8):957-71. PubMed ID: 17353235 [TBL] [Abstract][Full Text] [Related]
11. Studies on a sphingolipid activator protein (SAP-2) in fibroblasts from patients with lysosomal storage diseases, including Niemann-Pick disease Type C. Fujibayashi S; Wenger DA Clin Chim Acta; 1985 Mar; 146(2-3):147-56. PubMed ID: 3921288 [TBL] [Abstract][Full Text] [Related]
12. Lipid changes in Niemann-Pick disease type C brain: personal experience and review of the literature. Vanier MT Neurochem Res; 1999 Apr; 24(4):481-9. PubMed ID: 10227680 [TBL] [Abstract][Full Text] [Related]
13. Intracellular degradation of fluorescent glycolipids by lysosomal enzymes and their activators. Madar-Shapiro L; Pasmanik-Chor M; Dinur T; Dagan A; Gatt S; Horowitz M J Inherit Metab Dis; 1999 Jun; 22(5):623-37. PubMed ID: 10399095 [TBL] [Abstract][Full Text] [Related]
14. Characterization of Sellin J; Schulze H; Paradis M; Gosejacob D; Papan C; Shevchenko A; Psathaki OE; Paululat A; Thielisch M; Sandhoff K; Hoch M Dis Model Mech; 2017 Jun; 10(6):737-750. PubMed ID: 28389479 [TBL] [Abstract][Full Text] [Related]
15. Degradation of membrane-bound ganglioside GM1. Stimulation by bis(monoacylglycero)phosphate and the activator proteins SAP-B and GM2-AP. Wilkening G; Linke T; Uhlhorn-Dierks G; Sandhoff K J Biol Chem; 2000 Nov; 275(46):35814-9. PubMed ID: 10942779 [TBL] [Abstract][Full Text] [Related]
16. Lysosomal proteolysis of prosaposin, the precursor of saposins (sphingolipid activator proteins): its mechanism and inhibition by ganglioside. Hiraiwa M; Martin BM; Kishimoto Y; Conner GE; Tsuji S; O'Brien JS Arch Biochem Biophys; 1997 May; 341(1):17-24. PubMed ID: 9143348 [TBL] [Abstract][Full Text] [Related]
17. Saposins (sap) A and C activate the degradation of galactosylceramide in living cells. Harzer K; Paton BC; Christomanou H; Chatelut M; Levade T; Hiraiwa M; O'Brien JS FEBS Lett; 1997 Nov; 417(3):270-4. PubMed ID: 9409731 [TBL] [Abstract][Full Text] [Related]
18. 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; 50(6):1252-8. PubMed ID: 1350885 [TBL] [Abstract][Full Text] [Related]
19. 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 Biochim Biophys Acta; 2000 Nov; 1502(3):391-7. PubMed ID: 11068181 [TBL] [Abstract][Full Text] [Related]
20. Saposin C mutations in Gaucher disease patients resulting in lysosomal lipid accumulation, saposin C deficiency, but normal prosaposin processing and sorting. Vaccaro AM; Motta M; Tatti M; Scarpa S; Masuelli L; Bhat M; Vanier MT; Tylki-Szymanska A; Salvioli R Hum Mol Genet; 2010 Aug; 19(15):2987-97. PubMed ID: 20484222 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]