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Literature summary for 3.10.1.1 extracted from

  • Hemsley, K.M.; King, B.; Hopwood, J.J.
    Injection of recombinant human sulfamidase into the CSF via the cerebellomedullary cistern in MPS IIIA mice (2007), Mol. Genet. Metab., 90, 313-328.
    View publication on PubMed
Search for more literature for 3.10.1.1

Application

Application Comment Organism
medicine the enzyme might be an immediately applicable method for in vivo to treatment of lysosomal disorders like mucopolysaccharidosis type IIIA and similar lysosomal diseases in the central nervous system Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
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 Homo sapiens 5764
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Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens repeated human enzyme injection into murine cerebrospinal fluid via cisterna magna leads to a reduction of heparan sulfate-derived monosulfated disaccharide in the brain and spinal cord, reduced lysosomal vesicle formation in various cell types, reduced axonal spheroids, and improved behaviour of treated mice, mouse model, overview ?
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 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
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-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Homo sapiens
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information repeated human enzyme injection into murine cerebrospinal fluid via cisterna magna leads to a reduction of heparan sulfate-derived monosulfated disaccharide in the brain and spinal cord, reduced lysosomal vesicle formation in various cell types, reduced axonal spheroids, and improved behaviour of treated mice, mouse model, overview Homo sapiens ?
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 ?

Synonyms

Synonyms Comment Organism
SGSH
-
 Homo sapiens
sulfamidase
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 Homo sapiens