Medical Definition of I-cell disease

1. Mucolipidosis of early onset and with severe symptoms like those in Hurler's syndrome but with normal urinary mucopolysaccharides, vacuolated lymphocytes, and inclusion bodies in cultured fibroblasts (I-cells). The lysosomes lack hydrolases but high concentrations of lysosomal enzymes are found in the extracellular fluids such as serum, spinal fluid, and urine. It is associated with a deficiency of N-acetylglucosaminyl-1-phosphotransferase. The gene defect responsible probably prevents the addition of the lysosome recognition marker mannose 6 phosphate) to these enzymes so that they are not directed into the lysosomes but are released. Inheritance: autosomal recessive. Synonym: I-cell disease, inclusion cell disease. (12 Jul 2000)

Lexicographical Neighbors of I-cell Disease

I've
I've been robbed
I've been shot
I've burned myself
I've got your nose
I've lost my keys
I've never heard it called that before
I'ven't
I-HAWK
I-TEVII endonuclease
I-TEVI endonuclease
I-beam
I-beams
I-cell
I-cell disease (current term)
I-frame
I-frames
I-spin
I-spy
I.D.
I.Q.
I.W.W.
I. A. Richards
I. F. Stone
I. M. Pei
I/O
I/O bound
I2
I3

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