F
IPR016699

Acid ceramidase-like

InterPro entry
Short nameAcid_ceramidase-like

Description

This group represents acid ceramidases and some related proteins of currently unknown function. Ceramide is hydrolyzed by both acid and alkaline ceramidase
[1]
. Acid ceramidase requires saposin D, a sphingolipid activator protein, for the lysosomal breakdown of ceramide to a fatty acid and sphingosine
[2]
. Ceramide is found in plasma membranes and plays a pivotal role in the metabolism of sphingolipids. In addition, ceramide and sphingosine may have important roles as second messengers for cell function and survival
[1]
.

References

1.Stimulation of acid ceramidase activity by saposin D. Azuma N, O'Brien JS, Moser HW, Kishimoto Y. Arch. Biochem. Biophys. 311, 354-7, (1994). View articlePMID: 8203897

2.Structures of the human ceramide activator protein saposin D. Popovic K, Prive GG. Acta Crystallogr. D Biol. Crystallogr. 64, 589-94, (2008). View articlePMID: 18453694

Further reading

3. Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease. Koch J, Gartner S, Li CM, Quintern LE, Bernardo K, Levran O, Schnabel D, Desnick RJ, Schuchman EH, Sandhoff K. J. Biol. Chem. 271, 33110-5, (1996). PMID: 8955159

4. Spinal muscular atrophy associated with progressive myoclonic epilepsy is caused by mutations in ASAH1. Zhou J, Tawk M, Tiziano FD, Veillet J, Bayes M, Nolent F, Garcia V, Servidei S, Bertini E, Castro-Giner F, Renda Y, Carpentier S, Andrieu-Abadie N, Gut I, Levade T, Topaloglu H, Melki J. Am. J. Hum. Genet. 91, 5-14, (2012). PMID: 22703880

GO terms

Cross References

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