The uronic acid level in urine is elevated in -
Ans. is 'd' i.e. Mucopolysaccharidoses: Mucopolysaccharidosesare hereditary disorders caused by a deficiency of one of the lysosomal hydrolases involved in degradation of one or more of the glycosaminoglycans (GAGs also called as mucopolysaccharides).This results in the accumulation of GAGs in various trisaccharides).This results in the accumulation of GAGs in various tissues of the body. GAGs, as well as their oligosaccharides, are excreted in urine.GAGs are long, heteropolysaccharide chains generally composed of a repeating disaccharide unit .The amino sugar is either --D - glucosamine orD - galactosamineThe acidic sugar is either --D - glucuronic acid orL - Iduronic acid.There is increased excretion of this D - glucuronic acid or L - iduronic acid in urine in urine in mucopolysaccharidoses.There are many types of mucopolysaccharidoses of which the following are imp.Name - Enzyme defectHurler's syndrome - L- iduronidase*Hunter syndrome - Iduronate sulfatase*Morquio A syndrome - Galactosamine - 6 - sulfatase*Morquio B syndrome - B galactosidase*
Tyrosinosis
Maple syrup urine disease
Nieman picks disease
Mucopolysaccharidosis.