Mucolipidosis

One of a group of inherited storage diseases in which both lipids and substances called mucopolysaccharides accumulate in the tissues of the body.</P> Four mucolipidosis have been identified:</P> > >Mucolipidosis I: due to deficiency of the enzyme muramidase.

>Mucolipidosis II: due to deficiency of the enzyme UDP-N-acetylglucosamine-lysosomal-enzyme N-acetylglucosamine-phosphotransferase.

Mucolipidosis II is also called I-cell disease.

>Mucolipidosis III: due to deficiency of the enzyme N-acetylglucosamine-1-phosphotransferase.

Mucolipidosis III is also called pseudo-Hurler polydystrophy.

>Mucolipidosis IV: due to mutation in the gene encoding mucolipin-1. Mucolipidosis IV is also called sialolipidosis.

</LI></UL> All four of the mucolipidoses are lysosomal disorders -- the lysomes are organelles within the cell containing enzymes that can digest (lyse) substances -- and all are inherited in an autosomal recessive manner.