Sphingolipids activator protein

Niemann-Pick disease C type 2 Gmj activator protein deficiency Sphingolipid activator protein deficiency... 

Linke, T., Wilkening, G., Sadeghlar,. F, MozcaU, H., Bernardo, K., Schuchman, E., and Sandhoff, K., 2001, Interfacial Regulation of Acid Ceramidase Activity. Stimulation of ceramide degradation by lysosomal lipids and Sphingolipid Activator Proteins. J. Biol. Chem. 276 5760-5768. 

Kolter T, Sandhoff (2005) Principles of lysosomal membrane digestion stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids. Annu Rev Cell Dev Biol 21 81-103... 

Ferlinz, K., linke, T., Bartelsen, O., Weiler, M., and Sandhoff, K., 1999, Stimulation of lysosomal sphingomyelin degradation by sphingolipid activator proteins. Chem Phys Lipids 102 35-43. 

SAP Sphingolipid activator protein also serum amyloid P, shrimp alkaline... 

Klein A, Henseler M, Klein C, Suzuki K, Harzer K, Sandhoff K. Sphingolipid activator protein D (sap-D) stimulates the lysosomal degradation of ceramide in vivo. Biochem. Biophys. Res. 152. Commun. 1994 200 1440-1448. 

Zschoche A, Furst W, Schwarzmann G, San-hoff K (1994) Hydrolysis of lactosylceramide by human galacrtosylceramidase and GM1-/1-galactosidase in a detergent-free system and its stimulation by sphingolipid activator protein, sap-B and sup-C. Activator proteins stimulate lactosylceramide hydrolysis. Eur J Biochem 222 83-90... 

K. Sandhoff, T. Kelter, and G. Van Echten-Deckert Sphingolipid metabolism. Sphingoid analogs, sphingolipid activator proteins, and the pathology of the cell. Anna/i of the New YorkAcademy of Sciences 845 139 (1998). 

Wenger, D. A., and Inui, K., Studies on the sphingolipid activator protein for the enzymatic hydrolyses of CMj ganglioside and sulfatide. In Molecular Basis of Lysosomal Storage Disorders (J. A. Barranger and R. O. Brady, eds.), pp. 61-78. Academic Press, Orlando, 1984. 

Colsch et al., 2007). Because the diagnosis may be complicated in cases of arylsulfatase A pseudodeficiency and sphingolipid activator protein deficiency, this measurement of sulfatide in the urinary sediment of affected individuals by a rapid, sensitive, and specific mass spectrometric method has been long wanted (Whitfield et al., 2001). Urinary sulfatides are now commonly detected using electrospray ionization-tandem mass spectrometry by means of the precursor ion scan 97. Levels are considerably increased to X20-30 folds as compared to controls which allows the rapid screening of a large number of samples. 

The lysosomal hydrolysis of SM to Cer and phosphocholine is catalyzed by acid SMase, a water-soluble, lysosomal glycoprotein that interacts with a sphingolipid-activator protein (SAP) and anionic lipids such as bis(monoacylglycerol)phosphate (BMP) to hydrolyze interfacial SM (T. Linke, 2001). Acid SMase possesses an N-terminal SAP-homology domain that appears to stabilize the correctly folded form of acid SMase as well as to facilitate the interfacial interaction with substrate in vivo (M. Kolzer, 2004). Acidic SMase is also secreted by many types of cells (I. Tabas, 1999 I. Takahashi, 2005). 

N-acetylneuraminic acid N-glycolylneuraminic acid palmitoyl-CoA sphingosine 1-phosphate sphinganine 1-phosphate sphingolipid-activator protein serine... 

Figure 7. Lysosomal degradation of selected glycosphingolipids. The sphingolipid activator proteins necessary for in vivo degradation are indicated. SAP, sphingolipid activator protein.

Sphingolipid-activator protein deficiencies GM2-activator, pSAP, SAP-B, or SAP-C Different types... 

Matsuda, J, Vanier, MT, Saito, Y, Tohyama, J, Suzuki, K and Suzuki, K (2001) A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse. Hum Mol Genet, 10, 1191-1199. 

Sandhoff, K, Kolter, T and Harzer, K (2001) Sphingolipid activator proteins. In The Metabolic and Molecular Bases of Inherited Disease (CR Scriver, AL Beaudet, WS Sly and D VaUe, eds), Vol.in, 8th edn, McGraw-Hill, New York, pp.3371-3388. 

The sphingolipid activator proteins known to date are encoded by just two genes one carries the information for the GM2-activator [14] and the other for the so-called SAP-precursor or prosaposin, which is proteolytically processed to four ho-... 

Figure 4. Degradation of selected sphingolipids in the lysosomes of the cells [49]. The eponyms of individual inherited diseases (in frame) are given. Those activator proteins which are required for the respective degradation step in vivo are indicated. Variant AB, AB variant of GM2 gangliosidosis (deficiency of GM2-activator protein) SAP, Sphingolipid-activator protein.

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