Membrane traffic in sphingolipid storage diseases

Traffic

Volumn 1, Issue 11, 2000, Pages 807-815

  Pagano, Richard E ^a   Puri, Vishwajeet ^a   Dominguez, Michel ^a   Marks, David L ^a  

^a MAYO CLINIC   (United States)

Author keywords

Cholesterol; Endocytosis; Golgi apparatus; Membrane fluidity; Metabolic disorders; Microdomains; NPC1 protein

Indexed keywords

4,4 DIFLUORO 4 BORA 3A,4A DIAZA S INDACENE; 4,4-DIFLUORO-4-BORA-3A,4A-DIAZA-S-INDACENE; BORON DERIVATIVE; CHOLESTEROL; FLUORESCENT DYE; MEMBRANE LIPID; SPHINGOLIPID;

ACTIVE TRANSPORT; BIOLOGICAL MODEL; ENDOCYTOSIS; HUMAN; LIPIDOSIS; METABOLISM; REVIEW;

BIOLOGICAL TRANSPORT, ACTIVE; BORON COMPOUNDS; CHOLESTEROL; ENDOCYTOSIS; FLUORESCENT DYES; HUMANS; MEMBRANE LIPIDS; MODELS, BIOLOGICAL; SPHINGOLIPIDOSES; SPHINGOLIPIDS;

EID: 0034329025     PISSN: 13989219     EISSN: None     Source Type: Journal    
DOI: 10.1034/j.1600-0854.2000.011101.x     Document Type: Article

References (19)

1. Lysosomal enzymes. In Scriver CR, Beaudet AL, Sly WS, Valle D (Eds.), The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill, 1995: 2427-2839.
2. Neufeld EF. Lysosomal storage diseases. Ann Rev Biochem 1991;60: 257-280.
3. Sweeley CC. Sphingolipids. In Neuberger A, van Deenen LLM (Eds.), New Comprehensive Biochemistry. London: Elsevier, 1991: 327-361.
4. Fürst W, Sandhoff K. Activator proteins and topology of lysosomal sphingolipid catabolism. Rev Biochim Biophys Acta 1992;1126: 1-16.
5. Sandhoff K, Harzer K, Fürst W. Sphingolipid activator proteins. In Scriver CR, Beaudet AL, Sly WS, Valle D (Eds.), The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill, 1995: 2427-2441.
6. Bach G, Ziegler M, Kohn G, Cohen M. Mucopolysaccharide accumulation in cultured skin fibroblasts derived from patients with mucolipidosis IV. Am J Hum Genet 1977;29: 610-618.
7. Chen C, Bach G, Pagano R. Abnormal transport along the lysosomal pathway in mucolipidosis, type IV disease. Proc Natl Acad Sci USA 1998;95: 6373-6378.
8. Pentchev PG, Vanier MT, Suzuki K, Patterson MC. Niemann-Pick disease type C: a cellular cholesterol lipidosis. In Scriver CR, Beaudet AL, Sly WS, Valle D (Eds.), The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill, 1995: 2625-2639.
9. Chen CS, Patterson MC, Wheatley CL, O'Brien JF, Pagano RE. A broad screening test for sphingolipid storage diseases based on alterations in lipid trafficking. Lancet 1999;354: 901-905.
10. Puri V, Watanabe R, Dominguez M, Sun X, Wheatley CL, Marks DL, et al. Cholesterol modulates membrane traffic along the endocytic pathway in sphingolipid storage diseases. Nat Cell Biol 1999;1:386-388.
11. Brown DA, London E. Structure and function of sphingolipid- and cholesterol-rich membrane rafts. J Biol Chem 2000;275: 17221-17224.
12. Simons K, Ikonen E. Functional rafts in cell membranes. Nature 1997;387: 569-572.
13. Hoekstra D, Kok J. Trafficking of glycosphingolipids in eukaryotic cells; sorting and recycling of lipids. Biochim Biophys Acta 1992;1113:277-294.
14. Pagano RE, Sleight RG. Defining lipid transport pathway in animal cells. Science 1985;229: 1051-1057.
15. Schwarzmann G, Sandhoff K. Metabolism and intracellular transport of glycosphingolipids. Biochemistry 1990;29: 10865-10871.
16. Johannes L, Gould B. Surfing on a retrograde wave: how does Shiga toxin reach the endoplasmic reticulum? Trends Cell Biol 1998;8: 158-162.
17. Sandvig K, van Deurs B. Endocytosis, intracellular transport, and cytotoxic action of Shiga toxin and ricin. Physiol Rev 1996;76: 949-966.
18. Pagano RE, Martin OC, Kang HC, Haugland RP. A novel fluorescent ceramide analog for studying membrane traffic in animal cells: accumulation at the Golgi apparatus results in altered spectral properties of the sphingolipid precursor. J Cell Biol 1991;113: 1267-1279.
19. Pagano RE, Watanabe R, Wheatley C, Dominguez M. Novel applications of BODIPY™-sphingolipid analogs to study lipid traffic and metabolism in cells. Methods Enzymol 2000;312: 523-534.