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Medical Term:

UDP-N-acetylglucosamine:lysosomal enzyme N-acetylglucosaminyl-1-phosphotransferase

Pronunciation: a-sē′til-glū′kō-sam′ēn-līsō-sōm′ăl en′zīm, a - sē′til - glū′kō - sam′in - il - fos′fō - trans′fĕr - ās

Definition: An enzyme that participates in the posttranslational modification of a number of lysosomal proteins; a deficiency or defect in this enzyme results in two forms of mucolipidoses, I-cell disease, and pseudo-Hurler polydystrophy.