Optical rotatory dispersion of mucopolysaccharides IV. Optical rotatory dispersion and circular dichroism of glycosaminoglycans and heparan sulfate fractions from the urine of patients with mucopolysaccharidosis (Hurler Syndrome)

• Published in: Biochimica et biophysica acta Vol. 222; no. 1; pp. 79 - 89
• Main Authors: Stone, Audrey L., Constantopoulos, George, Sotsky, Stuart M., Dekaban, Anatole
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 27.10.1970
• Subjects: Animals; Carbazoles; Carbohydrate Metabolism, Inborn Errors - urine; Chondroitin - urine; Chromatography, Gel; Circular Dichroism; Glycosaminoglycans - metabolism; Glycosaminoglycans - urine; Heparin - urine; Humans; Indoles; Intellectual Disability - urine; Molecular Weight; Mucopolysaccharidosis I - urine; Optical Rotatory Dispersion; Rats; Resorcinols; Sulfuric Acids - urine; Ultraviolet Rays; ORD; CD; C/O

Genetic defects in the Hurler syndrome lead to the excessive accumulation of dermatan and heparan sulfate in many tissues and to the increase in excretion of these compounds in the urine. Patients exhibit mental detorioration, dwarfism, hepatosplenomegaly, corneal opacities and skeletal deformities....