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Literature summary extracted from

  • Qiu, H.; Edmunds, T.; Baker-Malcolm, J.; Karey, K.P.; Estes, S.; Schwarz, C.; Hughes, H.; Van Patten, S.M.
    Activation of human acid sphingomyelinase through modification or deletion of C-terminal cysteine (2003), J. Biol. Chem., 278, 32744-32752.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
3.1.4.12 additional information purified rhASM can be activated in vitro by loss of the free thiol on the C-terminal cysteine via chemical modification, dimerization, or deletion of this amino acid residue Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.1.4.12 expression in CHO cells Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.1.4.12 C629S 5fold increase in activity, modification or deletion of the thiol group on the C-terminal cysteine leads to activation of rhASM Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
3.1.4.12 1.7
-
 2-(N-hexadecanoylamino)-4-nitrophenylphosphorylcholine 37°C, pH 5.5 Homo sapiens

Localization

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

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
3.1.4.12 Cu2+ 6fold increase in activity at 0.1 mM Homo sapiens
3.1.4.12 Zn2+ 5fold increase in activity at 0.1 mM Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.1.4.12 sphingomyelin + H2O Homo sapiens
-
 N-acylsphingosine + choline phosphate
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
3.1.4.12 Homo sapiens
-
 one form of Niemann-Pick disease is caused by a deficiency of acid sphingomyelinase
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.1.4.12 glycoprotein at least five of six potential glycosylation sites are utilized Homo sapiens

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
3.1.4.12 21.1
-
-
 Homo sapiens
3.1.4.12 92.8
-
 5fold higher specific activity of rhASM purified from CHO cell-harvests stored at -20°C Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.1.4.12 2-(N-hexadecanoylamino)-4-nitrophenylphosphorylcholine + H2O
-
 Homo sapiens 2-(N-hexadecanoylamino)-4-nitrophenol + choline phosphate
-
 ?
3.1.4.12 sphingomyelin + H2O
-
 Homo sapiens N-acylsphingosine + choline phosphate
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.1.4.12 acid sphingomyelinase
-
 Homo sapiens
3.1.4.12 rhASM
-
 Homo sapiens