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Literature summary extracted from

  • Koelzer, M.; Ferlinz, K.; Bartelsen, O.; Hoops, S.L.; Lang, F.; Sandhoff, K.
    Functional characterization of the postulated intramolecular sphingolipid activator protein domain of human acid sphingomyelinase (2004), Biol. Chem., 385, 1193-1195.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
3.1.4.12 saposin C presence of sphingolipid activator proteins, e. g. saponsins, is not essential for activity. Saposin C increases activity of the P153A mutant enzyme, but does not result in complete restoration of the activity Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.1.4.12 C120S mutant enzyme is almost completely devoid of activity in the detergent-containing micellar assay system as well as in the liposomal assay system in the presence of saposin C Homo sapiens
3.1.4.12 C131S mutant enzyme is almost completely devoid of activity in the detergent-containing micellar assay system as well as in the liposomal assay system in the presence of saposin C Homo sapiens
3.1.4.12 K118E mutant enzyme is almost completely devoid of activity in the detergent-containing micellar assay system as well as in the liposomal assay system in the presence of saposin C Homo sapiens
3.1.4.12 O153A mutant enzyme still exhibits enzyme activity of approximately 52% of normal in a detergent-containing micellar assay, but only 13% of normal activity in a detergent-free liposomal assay system. Addition of saposin C to the liposomal ass mixture increases activity of the mutant enzyme to 46% of normal. Saposin C does not result in complete restoration of the variant activity Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.1.4.12 lysosome
-
 Homo sapiens 5764
-

Organism

EC Number Organism UniProt Comment Textmining
3.1.4.12 Homo sapiens
-
-
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.1.4.12 sphingomyelin + H2O the sap-like domain of human acid sphingomyelinase has activator-like functions, as well as importance as an integral part of acid spingomyelinase required for normal enzyme activity Homo sapiens N-acylsphingosine + choline phosphate
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.1.4.12 A-SMase
-
 Homo sapiens
3.1.4.12 acid sphingomyelinase
-
 Homo sapiens