Purification, characterization, and biosynthesis of human acid ceramidase.

  title={Purification, characterization, and biosynthesis of human acid ceramidase.},
  author={Katussevani Bernardo and Robert Hurwitz and T S Zenk and Robert J Desnick and Klaus Ferlinz and Edward H Schuchman and Konrad Sandhoff},
  journal={The Journal of biological chemistry},
  volume={270 19},
Acid ceramidase (N-acylsphingosine deacylase, EC is the lysosomal enzyme catalyzing the hydrolysis of ceramide to sphingosine and free fatty acid. Its inherited deficiency causes ceramide accumulation in Farber's disease. The enzyme was purified to apparent homogeneity from human urine by sequential chromatography on octyl-Sepharose, concanavalin A-Sepharose, blue-Sepharose, and DEAE-cellulose. The final preparation, which was enriched approximately 4450-fold over the starting… CONTINUE READING


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