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Literature summary extracted from

  • Kloska, A.; Bohdanowicz, J.; Konopa, G.; Tylki-Szym?ska, A.; Jakobkiewicz-Banecka, J.; Czartoryska, B.; Liberek, A.; Wegrzyn, A.; Wegrzyn, G.
    Changes in hair morphology of mucopolysaccharidosis I patients treated with recombinant human alpha-L-iduronidase (laronidase, Aldurazyme) (2005), Am. J. Med. Genet. A, 139, 199-203.
    View publication on PubMed

Application

EC Number Application Comment Organism
3.2.1.76 pharmacology the enzyme is used in enzyme replacement therapy of mucopolysaccharidosis type I, MPSI, changes in hair morphology of MPSI patients treated with recombinant human enzyme, overview Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.2.1.76 additional information nonsense mutation of residue Q70 is involved in the disease mucopolysaccharidosis type I, MPSI, changes in hair morphology of MPSI patients treated with recombinant human enzyme in enzyme replacement therapy, overview Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.2.1.76 additional information Homo sapiens enzyme deficiency causes mucopolysaccharidosis type I, MPSI ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.76 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.76 leukocyte
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.76 4-nitrophenyl-alpha-L-iduronide + H2O
-
 Homo sapiens 4-nitrophenol + alpha-L-iduronic acid
-
 ?
3.2.1.76 additional information enzyme deficiency causes mucopolysaccharidosis type I, MPSI Homo sapiens ?
-
 ?