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Literature summary for 3.5.1.15 extracted from

  • Poddar, N.K.; Zano, S.; Natarajan, R.; Yamamoto, B.; Viola, R.E.
    Enhanced brain distribution of modified aspartoacylase (2014), Mol. Genet. Metab., 113, 219-224.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.5.1.15

Application

Application Comment Organism
pharmacology the enzyme is the taget for treatment of Canavan disease, enzyme replacement therapy can potentially be used to overcome these defects if a stable enzyme form that can gain access to the appropriate neural cells can be produced. PEGylated form of aspartoacylase are able to traverse the blood-brain barrier and show dramatic enhancement in brain tissue access and distribution, overview. Examination of the effect of enzyme administration on the immunological response Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
gene acy2, recombinant expressionin Pichia pastoris strain KM71H Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information synthesis of PEGylated enzyme by treatment of enzyme samples with amethoxy-PEG reagent containing terminal activating aldehyde or ester groups attached with a carboxymethyl linker, purification of the protein-PEG conjugates by anion exchange chromatography, labeling with a covalently attached fluorescent tag, method optimization, overview Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens P45381 gene acy2 or ASPA
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Source Tissue

Source Tissue Comment Organism Textmining
brain
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 Homo sapiens
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Synonyms

Synonyms Comment Organism
ASPA
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction mutations in the ASPA gene cause the Canavan disease, a fatal, childhood neurological disorder leading to catalytic deficiencies in the aspartoacylase enzyme and impaired N-acetyl-L-aspartic acid metabolism in the brain. Enzyme replacement therapy can potentially be used to overcome these defects if a stable enzyme form that can gain access to the appropriate neural cells can be produced. Achieving the proper cellular targeting requires a modified form of aspartoacylase that can traverse the blood-brain barrier Homo sapiens