Tracking sphingosine metabolism and transport in sphingolipidoses: NPC1 deficiency as a test case.

@article{Blom2012TrackingSM,
  title={Tracking sphingosine metabolism and transport in sphingolipidoses: NPC1 deficiency as a test case.},
  author={Tomas Blom and Zaiguo Li and Robert Bittman and Pentti Somerharju and Elina Ikonen},
  journal={Traffic},
  year={2012},
  volume={13 9},
  pages={1234-43}
}
The late endosomal/lysosomal compartment (LE/LY) plays a key role in sphingolipid breakdown, with the last degradative step catalyzed by acid ceramidase. The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein (CERT) to the Golgi for conversion to sphingomyelin. The mechanism by which sphingosine exits LE/LY is unknown but Niemann-Pick C1 protein (NPC1) has been suggested to be involved. Here, we used sphingomyelin, ceramide and sphingosine… CONTINUE READING

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Here , we used sphingomyelin , ceramide and sphingosine labeled with [ ( 3)H ] in carbon-3 of the sphingosine backbone and targeted them to LE / LY in low - density lipoprotein ( LDL ) particles .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
Here , we used sphingomyelin , ceramide and sphingosine labeled with [ ( 3)H ] in carbon-3 of the sphingosine backbone and targeted them to LE / LY in low - density lipoprotein ( LDL ) particles .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
Here , we used sphingomyelin , ceramide and sphingosine labeled with [ ( 3)H ] in carbon-3 of the sphingosine backbone and targeted them to LE / LY in low - density lipoprotein ( LDL ) particles .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
Here , we used sphingomyelin , ceramide and sphingosine labeled with [ ( 3)H ] in carbon-3 of the sphingosine backbone and targeted them to LE / LY in low - density lipoprotein ( LDL ) particles .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
The released sphingosine can be converted to ceramide in the ER and transported by ceramide transfer protein ( CERT ) to the Golgi for conversion to sphingomyelin .
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