mucolipidosis

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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.

mu·co·lip·i·do·sis

, pl.

mu·co·lip·i·do·ses

(myū'kō-lip-i-dō'sis, -sēz),
Any of a group of lysosomal storage diseases in which symptoms of visceral and mesenchymal mucopolysaccharide, glycoprotein, oligosaccharide, or glycolipid storage are present; clinically, they bear a superficial resemblance to the mucopolysaccharidoses; autosomal recessive inheritance.
[muco- + lipid + -osis, condition]

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.

mucolipidosis

(myo͞o′kō-lĭp′ĭ-dō′sĭs)
n.
Any of a several progressive genetic diseases in which glycosaminoglycans and lipids accumulate in the tissues (without excretion of glycosaminoglycans in the urine), usually appearing in infancy or early childhood and resulting in symptoms of varied severity, often including musculoskeletal abnormalties and intellectual disability.

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

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

mu·co·lip·i·do·sis

, pl. mucolipidoses (myū'kō-lip-i-dō'sis, -sēz)
Any of a group of lysosomal storage diseases in which symptoms of visceral and mesenchymal mucopolysaccharide, glycoprotein, oligosaccharide, or glycolipid storage are present.
[muco- + lipid + -osis, condition]

mu·co·lip·i·do·sis

, pl. mucolipidoses (myū'kō-lip-i-dō'sis, -sēz)
A lysosomal storage disease in which symptoms of visceral and mesenchymal mucopolysaccharide, glycoprotein, oligosaccharide, or glycolipid storage are present.
[muco- + lipid + -osis, condition]

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.
References in periodicals archive ?
In light of the recent ruling that AIG did not assign its fraud claims to ML II, we have agreed to dismiss our declaratory judgment action, without prejudice to our right to reinstitute it if necessary," AIG spokesman James Ankner said, referring to the New York case.
Specifically, Ankner said, AIG "narrowly" seeks a declaration from the Court that a 2008 contract between AIG and ML II did not transfer to ML II AIG's right to sue Bank of America and other financial institutions for the billions of dollars of damages they caused AIG and its shareholders in connection with the fraudulent sale of RMBS to AIG.
This transaction substantially reduces the ML II portfolio and loan at a desirable price.
Conclusion: Treatment of pressed mistletoe juice with N-acetyl-galactosamine prior to lectin determination avoids or at least strongly reduces the interference of ML II and III in standard ML I assays (ELLA) and thus provides a simple method to evaluate the content of ML 1 more exactly.
2,4,6) However, now it is evident that only ML II and ML III are related allelic disorders of lysosomal trafficking.
It should be noted that the $152 billion decrease only includes a reduction of original authorized support and not the FRBNY's profit on ML II or the profit to date from ML III.
In that declaration, Mahoney asserted that "[t]he FRBNY and ML II intended for ML II to receive all transferable and assignable benefits associated with the securities and related instruments, including litigation claims associated with those securities or their acquisition by AIG.
contains three groups of glycosylated isolectins of the RIP II typ, mistletoe lectin I ( ML I), ML II and ML III, which recognize sugars and glycoconjugates containing galactose and/or N-acetyl-galactosamine groups.
It should be noted that the $152 billion decrease only includes a reduction of original authorized support and not the Federal Reserve's profit to date on ML II or the expected profit for ML III.
James Ankner, a spokesman for AIG corporate, says AIG is seeking a declaration from the court that the 2008 contract between AIG and ML II did not transfer to ML II AIG's right to sue Bank of America and other financial institutions for billions of dollars in damages they allegedly caused AIG and its shareholders in connection with the fraudulent sale of residential mortgage-backed securities (RMBS) to AIG.
The lectins ML I, ML II and ML III share the typical A-S-S-B structure of RIP II and recognize galactoside/N-acetyl-galactosaminide containing glycoconjugates.
FRBNY extended a senior loan to ML II to enable the purchase of the RMBS for an initial purchase price of $19.