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5. 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]
6. Importance of splicing for prosaposin sorting. Madar-Shapiro L; Pasmanik-Chor M; Vaccaro AM; Dinur T; Dagan A; Gatt S; Horowitz M Biochem J; 1999 Feb; 337 ( Pt 3)(Pt 3):433-43. PubMed ID: 9895286 [TBL] [Abstract][Full Text] [Related]
7. Synthesis of novel NBD-GM1 and NBD-GM2 for the transfer activity of GM2-activator protein by a FRET-based assay system. Schwarzmann G; Wendeler M; Sandhoff K Glycobiology; 2005 Dec; 15(12):1302-11. PubMed ID: 16079415 [TBL] [Abstract][Full Text] [Related]
8. Substrate binding properties of the human liver hexosaminidase A activator protein. Hechtman P; Isaacs C; Smith-Jones L Can J Biochem Cell Biol; 1985 Aug; 63(8):830-8. PubMed ID: 2933135 [TBL] [Abstract][Full Text] [Related]
9. An Asn > Lys substitution in saposin B involving a conserved amino acidic residue and leading to the loss of the single N-glycosylation site in a patient with metachromatic leukodystrophy and normal arylsulphatase A activity. Regis S; Filocamo M; Corsolini F; Caroli F; Keulemans JL; van Diggelen OP; Gatti R Eur J Hum Genet; 1999; 7(2):125-30. PubMed ID: 10196694 [TBL] [Abstract][Full Text] [Related]
10. Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse. Matsuda J; Kido M; Tadano-Aritomi K; Ishizuka I; Tominaga K; Toida K; Takeda E; Suzuki K; Kuroda Y Hum Mol Genet; 2004 Nov; 13(21):2709-23. PubMed ID: 15345707 [TBL] [Abstract][Full Text] [Related]
11. 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]
13. 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]
14. The biochemical basis of gangliosidoses. Sandhoff K; Conzelmann E Neuropediatrics; 1984 Sep; 15 Suppl():85-92. PubMed ID: 6242704 [No Abstract] [Full Text] [Related]
15. Activator proteins and topology of lysosomal sphingolipid catabolism. Fürst W; Sandhoff K Biochim Biophys Acta; 1992 Jun; 1126(1):1-16. PubMed ID: 1606169 [TBL] [Abstract][Full Text] [Related]
16. Processing of sphingolipid activator proteins and the topology of lysosomal digestion. Sandhoff K; Kolter T Acta Biochim Pol; 1998; 45(2):373-84. PubMed ID: 9821868 [TBL] [Abstract][Full Text] [Related]
17. Deficiency of the hexosaminidase A activator protein in a case of GM2 gangliosidosis; variant AB. Hechtman P; Gordon BA; Ng Ying Kin NM Pediatr Res; 1982 Mar; 16(3):217-22. PubMed ID: 6801612 [TBL] [Abstract][Full Text] [Related]
18. Modulation of human saposin B sphingolipid-binding specificity by alternative splicing. A study with saposin B-derived synthetic peptides. Lamontagne S; Potier M J Biol Chem; 1994 Aug; 269(32):20528-32. PubMed ID: 8051151 [TBL] [Abstract][Full Text] [Related]
19. Distribution of saposins (sphingolipid activator proteins) in tissues of lysosomal storage disease patients. Tayama M; O'Brien JS; Kishimoto Y J Mol Neurosci; 1992; 3(4):171-5. PubMed ID: 1389998 [TBL] [Abstract][Full Text] [Related]
20. Structural organization and expression of the gene for the mouse GM2 activator protein. Bertoni C; Appolloni MG; Stirling JL; Li SC; Li YT; Orlacchio A; Beccari T Mamm Genome; 1997 Feb; 8(2):90-3. PubMed ID: 9060405 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]