Enzyme Composition Synthesis and Biochemical Action 12211 Protein and Gene Sequence and Structure

The coding sequence of human a-L-iduronidase encodes a 653-amino acid protein derived from a gene consisting of 14 exons and covering about 20 kb of sequence on chromosome 4p, close to the Huntington disease locus [15]. The published sequence is one of the many polymorphic forms of the iduronidase enzyme that are found in the human population [16]. The sequence used in the production of Aldurazyme contains one of these polymorphisms, in which a glutamine at position 33 (or position 8 in the mature protein) is replaced

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