Mucoproteins. Conjugated proteins in which mucopolysaccharides are combined with proteins. The mucopolysaccharide moiety is the predominant group with the protein making up only a small percentage of the total weight. . 1.00
Glycosaminoglycans. Mucopolysaccharides . Heteropolysaccharides which contain an N-acetylated hexosamine in a characteristic repeating disaccharide unit. The repeating structure of each disaccharide involves alternate 1,4- and 1,3-linkages consisting of either N-acetylglucosamine (see ACETYLGLUCOSAMINE) or N-acetylgalactosamine (see ACETYLGALACTOSAMINE). . 0.59
Mucopolysaccharidoses. Mucopolysaccharidosis . Group of lysosomal storage diseases each caused by an inherited deficiency of an enzyme involved in the degradation of glycosaminoglycans (mucopolysaccharides). The diseases are progressive and often display a wide spectrum of clinical severity within one enzyme deficiency. . 0.54
Mucopolysaccharidosis I. Gargoylism . Gargoylism, Hurler Syndrome . Hurler Disease . Hurler Syndrome . Hurler's Disease . Mucopolysaccharidosis 1 . Mucopolysaccharidosis 5 . Mucopolysaccharidosis I-S . Mucopolysaccharidosis Type I . Mucopolysaccharidosis Type Ih . Mucopolysaccharidosis Type Ih S . Mucopolysaccharidosis Type Is . Scheie Syndrome . alpha-L-Iduronidase Deficiency . Disease, Hurler's . Gargoylisms . Hurler Scheie Syndrome . Hurler Syndrome Gargoylism . Lipochondrodystrophies . Mucopolysaccharidosis I S . Mucopolysaccharidosis Is . Mucopolysaccharidosis Type Ihs . Syndrome, Hurler's . Syndrome, Scheie's . Type Ih, Mucopolysaccharidosis . Type Ihs, Mucopolysaccharidosis . alpha L Iduronidase Deficiency . alpha-L-Iduronidase Deficiencies . Hurler's Syndrome . Pfaundler-Hurler Syndrome . Lipochondrodystrophy . Hurler-Scheie Syndrome . Mucopolysaccharidosis V . Scheie's Syndrome . GARGOYLISM . Systemic lysosomal storage disease caused by a deficiency of alpha-L-iduronidase (IDURONIDASE) and characterized by progressive physical deterioration with urinary excretion of DERMATAN SULFATE and HEPARAN SULFATE. There are three recognized phenotypes representing a spectrum of clinical severity from severe to mild: Hurler syndrome, Hurler-Scheie syndrome and Scheie syndrome (formerly mucopolysaccharidosis V). Symptoms may include DWARFISM; hepatosplenomegaly; thick, coarse facial features with low nasal bridge; corneal clouding; cardiac complications; and noisy breathing. . 0.53
Glycopeptides. Proteins which contain carbohydrate groups attached covalently to the polypeptide chain. The protein moiety is the predominant group with the carbohydrate making up only a small percentage of the total weight. . 0.52
Mucopolysaccharidosis II. Hunter Syndrome . Hunter Syndrome Gargoylism . I2S Deficiency . Mucopolysaccharidosis 2 . Mucopolysaccharidosis Type 2 . Mucopolysaccharidosis Type II . Deficiency, I2S . Deficiency, Iduronate 2-Sulfatase . Deficiency, Iduronate Sulfatase . Deficiency, Sulfoiduronate Sulfatase . Hunters Syndrome . Iduronate 2 Sulfatase Deficiency . Syndrome, Hunter . Syndrome, Hunter's . Gargoylism, Hunter Syndrome . Hunter's Syndrome . Iduronate 2-Sulfatase Deficiency . Iduronate Sulfatase Deficiency . Sulfoiduronate Sulfatase Deficiency . Systemic lysosomal storage disease marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This disease differs from MUCOPOLYSACCHARIDOSIS I by slower progression, lack of corneal clouding, and X-linked rather than autosomal recessive inheritance. The mild form produces near-normal intelligence and life span. The severe form usually causes death by age 15. . 0.49
