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Literature summary for 3.1.1.13 extracted from

  • Du, H.; Cameron, T.L.; Garger, S.J.; Pogue, G.P.; Hamm, L.A.; White, E.; Hanley, K.M.; Grabowski, G.A.
    Wolman disease/cholesteryl ester storage disease: efficacy of plant-produced human lysosomal acid lipase in mice (2008), J. Lipid Res., 49, 1646-1657.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Nicotiana benthamiana Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information genetic LAL mutations lead to Wolman disease and cholesteryl ester storage disease, plant-expressed recombinant human LAL can be used for enzyme therapy of human Wolman disease/cholesteryl ester storage disease Homo sapiens

Localization

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
butyl-Sepharose column chromatography and SP-Sepharose column chromatography Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl oleate + H2O
-
Homo sapiens 4-methylumbelliferol + oleate
-
?

Synonyms

Synonyms Comment Organism
LAL
-
Homo sapiens
lysosomal acid lipase
-
Homo sapiens