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Literature summary for 3.1.4.12 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

Activating Compound Comment Organism Structure
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)

Cloned (Comment) Organism
expression in CHO cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
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]

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

Localization

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

Metals/Ions

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

Natural Substrates/ Products (Substrates)

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

Organism

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

Posttranslational Modification

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

Specific Activity [micromol/min/mg]

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

Substrates and Products (Substrate)

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

Synonyms

Synonyms Comment Organism
acid sphingomyelinase
-
Homo sapiens
rhASM
-
Homo sapiens