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Literature summary for 3.1.4.12 extracted from

  • Ziegler, R.J.; Brown, C.; Barbon, C.M.; DAngona, A.M.; Schuchman, E.H.; Andrews, L.; Thurberg, B.L.; McPherson, J.M.; Karey, K.P.; Cheng, S.H.
    Pulmonary delivery of recombinant acid sphingomyelinase improves clearance of lysosomal sphingomyelin from the lungs of a murine model of Niemann-Pick disease (2009), Mol. Genet. Metab., 97, 35-42.
    View publication on PubMed

Application

Application Comment Organism
medicine systemic administration of recombinant acid sphingomyelinase (rhASM) into ASM deficient mice by intranasal instillation results in hydrolysis of the abnormal storage of sphingomyelin in lysosomes of the liver, spleen and lung, pulmonary administration of rhASM may represent an alternative route of delivery to address the visceral pathology associated with ASM deficiency Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining

Substrates and Products (Substrate)

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

Synonyms

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