mucolipidosis (redirected from Mucolipidoses)

mucolipidosis /mu·co·lip·i·do·sis/ (mu″ko-lip″ĭ-do´sis) pl. mucolipido´ses   any of a group of lysosomal storage diseases in which both glycosaminoglycans (mucopolysaccharides) and lipids accumulate in tissues but without excess of the former in the urine.

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mu·co·lip·i·do·sis (myk-lp-dss)

n.

Any of a group of hereditary metabolic storage diseases resembling Hurler's syndrome but with normal urinary mucopolysaccharides.

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mucolipidosis

[myo̅o̅′kōlip′idō′sis]

any of a group of metabolic disorders characterized by an accumulation of mucopolysaccharides and lipids in the tissues, but without an excess of mucopolysaccharides in the urine. Mucolipidosis includes I cell disease (mucolipidosis II) and pseudo-Hurler polydystrophy (mucolipidosis III).

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mucolipidosis [mu″ko-lip″ĭ-do´sis] (pl. mucolipido´ses)

any of a group of genetic disorders in which both glycosaminoglycans (GAGs) and lipids accumulate in tissues, but without excess of GAG in the urine.

mucolipidosis I sialidosis (type I).

mucolipidosis II a rapidly progressing disease of young children, histologically characterized by abnormal fibroblasts containing a large number of dark inclusions which fill the central part of the cytoplasm except for the juxtanuclear zone (I-cells), and clinically by severe growth impairment, minimal hepatic enlargement, extreme mental and motor retardation, and clear corneas; inherited as an autosomal recessive trait, it is due to deficiency of multiple lysosomal hydrolases. Called also I-cell disease.

mucolipidosis III a disorder similar to but milder than mucolipidosis II, and thought to be due to the same enzyme deficiency but to a lesser extent. Called also pseudo-Hurler polydystrophy.

mucolipidosis IV a form marked by early corneal clouding, psychomotor retardation, and the presence of lysosomal storage bodies; thought to be transmitted as an autosomal recessive trait.

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mucolipidosis

a group of inherited lysosomal storage diseases in which mucopolysaccharides and lipids accumulate in tissues. Mucolipidosis I is also called sialosis.

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mucolipidosis II

lysosomes contain large inclusions of undigested glycosaminoglycans and glycolipids. Called also I-cell disease.

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mucolipidosis

Any of 4 AR lysosomal storage diseases characterized by accumulation of glycolipids in interstitial tissues, which have clinical features similar to the mucopolysaccharidoses