Alkaline ceramidase 3

Definition:

Endoplasmic reticulum and Golgi ceramidase that catalyzes the hydrolysis of unsaturated long-chain C18:1-, C20:1- and C20:4-ceramides, dihydroceramides and phytoceramides into sphingoid bases like sphingosine and free fatty acids at alkaline pH. Ceramides, sphingosine, and its phosphorylated form sphingosine-1-phosphate are bioactive lipids that mediate cellular signaling pathways regulating several biological processes including cell proliferation, apoptosis and differentiation. Controls the generation of sphingosine in erythrocytes, and thereby sphingosine-1-phosphate in plasma. Through the regulation of ceramides and sphingosine-1-phosphate homeostasis in the brain may play a role in neurons survival and function (By similarity). By regulating the levels of pro-inflammatory ceramides in immune cells and tissues, may modulate the inflammatory response (By similarity).

References:

[1]. Ruijuan Xu, et al. Role of alkaline ceramidases in the generation of sphingosine and its phosphate in erythrocytes. FASEB J. 2010 Jul;24(7):2507-15. [Content Brief]

[2]. Simon Edvardson, et al. Deficiency of the alkaline ceramidase ACER3 manifests in early childhood by progressive leukodystrophy. J Med Genet. 2016 Jun;53(6):389-96. [Content Brief]

[3]. Ieva Vasiliauskaité-Brooks, et al. Structure of a human intramembrane ceramidase explains enzymatic dysfunction found in leukodystrophy. Nat Commun. 2018 Dec 21;9(1):5437. [Content Brief]

[4]. C Mao, et al. Cloning and characterization of a novel human alkaline ceramidase. A mammalian enzyme that hydrolyzes phytoceramide. J Biol Chem. 2001 Jul 13;276(28):26577-88. [Content Brief]

[5]. Wei Hu, et al. Alkaline ceramidase 3 (ACER3) hydrolyzes unsaturated long-chain ceramides, and its down-regulation inhibits both cell proliferation and apoptosis. J Biol Chem. 2010 Mar 12;285(11):7964-76. [Content Brief]

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