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mucolipidosis
(redirected from Mucolipidoses)

   Also found in: Wikipedia 0.02 sec.
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.
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.

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).

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.

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

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

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


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net/moe bius 1,2,3,6,8,9 MONOSOMY X See: Turner Syndrome MORQUIO SYNDROME See: Mucopolysaccharidosis; Tay-Sachs Disease MORVAN DISEASE See: Syringomyelia MOTOR-SENSORY NEUROPATHY, HEREDITARY See: Charcot-Marie-Tooth Disease MOYAMOYA DISEASE See: Stroke; Vascular Malformations MUCOLIPIDOSES See: Mucopolysaccharidosis; Mucolipidosis Type IV; Tay-Sachs Disease MUCOLIPIDOSIS TYPE IV See also: Mucopolysaccharidosis; Tay-Sachs Disease; Visual Impairments ML4 Foundation 719 East 17th St.
net/moebius/ 1,2,3,6,8,9 MONOSOMY X See: Turner Syndrome MORQUIO SYNDROME See: Mucopolysaccharidosis; Tay-Sachs Disease MORVAN DISEASE See: Syringomyelia MOTOR-SENSORY NEUROPATHY, HEREDITARY See: Charcot-Marie-Tooth Disease MOYAMOYA DISEASE See: Stroke; Vascular Malformations MUCOLIPIDOSES See: Mucopolysaccharidosis; Mucolipidosis Type IV; TaySachs Disease MUCOLIPIDOSIS TYPE IV See also: Mucopolysaccharidosis; TaySachs Disease; Visual Impairments ML4 Foundation 719 East 17th St.
This characteristic has not been described in other mucolipidoses or in the related mucopolysachharidoses and is thus an excellent diagnostic clue.
 
 
 
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