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morquio syndrom - in the body and brain which can lead to...

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Click to edit Master subtitle style 5/11/09 Morquio Syndrom Morquio syndrom is a genetic disorder that is autosomal recessive. It occurs in two types: type A and type B. Type A Morquio syndrom occurs when the body lacks a substance called galactosamine-6- sulfatase , and type B Morquio syndrom occurs when the body lacks an enzyme called beta-galactosidase. These two substances are enzymes that catalyze long sugar chains called keraten sulfate sugar chains or glycosaminoglycans . The shortage of these enzymes causes an excessive buildup of glycosaminoglycans
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Unformatted text preview: in the body and brain which can lead to organ damage. p-nitrophenyl-beta-galactoside, 4-methylumbelliferyl-beta-galactoside (4 MU-beta-gal), lactose, GM1 ganglioside, keratan sulfate, and asialofetuin (ASF) are some examples of these longs sugar chains. There is a 1 in 200,000 chance of a newborn to have this disorder because of the fact that it is autosomal recessive which means both your parents have to give you the gene that carries this disorder. 5/11/09 Beta-galactosidase lactose Galactosamine-6-sulfate sulfatase Glycosaminegly cans...
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