chondroitin and Mucopolysaccharidosis-IV
chondroitin has been researched along with Mucopolysaccharidosis-IV* in 40 studies
Reviews
6 review(s) available for chondroitin and Mucopolysaccharidosis-IV
Article | Year |
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Glycosaminoglycans. A biochemical and clinical review.
Topics: Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Chondroitin; Cornea; Corneal Opacity; Dermatan Sulfate; Fibroblasts; Galactose; Glucosamine; Glycosaminoglycans; Heparin; Humans; Hyaluronic Acid; Hyaluronoglucosaminidase; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Proteoglycans; Retinitis Pigmentosa; Skin; Staining and Labeling; Sulfatases; Testis | 1974 |
The genetic mucopolysaccharidoses (GMS).
Topics: Carbohydrate Metabolism, Inborn Errors; Chemical Phenomena; Chemistry; Chondroitin; Corneal Opacity; Gangliosides; Glycosaminoglycans; Histocytochemistry; Humans; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1973 |
[Biochemistry of mucopolysaccharidosis].
Topics: Chemical Phenomena; Chemistry; Chondroitin; Corneal Opacity; Female; Glucuronidase; Glycosaminoglycans; Humans; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1973 |
The systemic mucopolysaccharidoses.
Topics: Adolescent; Bone and Bones; Carbohydrate Metabolism, Inborn Errors; Child, Preschool; Chondroitin; Chromosome Aberrations; Chromosome Disorders; Female; Fetus; Glycolipids; Glycosaminoglycans; Humans; Hypertrichosis; Infant; Intellectual Disability; Joint Diseases; Male; Microscopy, Electron; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1972 |
[Urinary secretion of mucopolysaccharides under normal and pathological conditions].
Topics: Age Factors; Atmospheric Pressure; Burns; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Connective Tissue; Corneal Opacity; Glomerular Filtration Rate; Glycosaminoglycans; Humans; Intellectual Disability; Lupus Erythematosus, Systemic; Mucopolysaccharidosis IV; Neoplasms; Retinitis Pigmentosa | 1972 |
The nosology of the mucopolysaccharidoses.
Topics: Autopsy; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Chromosome Aberrations; Chromosome Disorders; Dwarfism; Fibroblasts; Glycosaminoglycans; Histocytochemistry; Humans; Hyaluronic Acid; Intellectual Disability; Joint Diseases; Metaplasia; Microscopy, Electron; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Osteochondritis; Retinitis Pigmentosa | 1969 |
Other Studies
34 other study(ies) available for chondroitin and Mucopolysaccharidosis-IV
Article | Year |
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Biochemical defect of non-keratan-sulfate-excreting Morquio syndrome.
Two children of second-cousin parents were found to have a very mild form of Morquio syndrome. The 14-year-old boy was 147 cm tall and had fine corneal deposits, a broad chest, dislocated hips, and flat feet. His 7-year-old sister had a broad chest but otherwise normal physical development. An abnormal lumbar spine was seen in radiographs of both children. Analysis of the urine from the affected children showed levels of acid mucopolysaccharides (AMPS) up to twice as high as that found in normal urine, but no evidence of keratosulfaturia. Most urinary AMPS was chondroitin-6-sulfate. Multiple assays of N-acetylgalactosamine-6-sulfate (GalNAc-6-SO4) sulfatase in leukocytes and cultured skin fibroblasts showed deficiency of this enzyme in the range found in the classical form of Morquio (Morquio A) syndrome. This report identifies an enzymatic defect in one form of non-keratan-sulfate-excreting Morquio (NKSE Morquio) syndrome and confirms the absence of keratosulfaturia in this mild form of Morquio disease. Topics: Adolescent; Child; Chondroitin; Chondroitin Sulfates; Consanguinity; Female; Fibroblasts; Genes, Recessive; Genetic Variation; Glycosaminoglycans; Humans; Keratan Sulfate; Leukocytes; Male; Mucopolysaccharidosis IV; Pedigree | 1983 |
Spondyloepiphyseal dysplasia, chondroitin sulfate type: a possible defect of PAPS--chondroitin sulfate sulfotransferase in humans.
Topics: Adenine Nucleotides; Animals; Chick Embryo; Chondroitin; Chondroitin Sulfates; Fibroblasts; Glycosaminoglycans; Humans; Mice; Mucopolysaccharidosis IV; Phosphoadenosine Phosphosulfate; Sulfotransferases; Sulfurtransferases | 1981 |
Recessively inherited, late onset spondylar dysplasia and peripheral corneal opacity with anomalies in urinary mucopolysaccharides: a possible error of chondroitin-6-sulfate synthesis.
Two male and two female sibs with an unusual form of spondyloepiphyseal dysplasia were reported. The main clinical features were low stature, moderate shortness of trunk and neck, abnormal span: height ratio, low-normal UBS: LBS ratio, and peripheral corneal punctate opacities only seen by the slitlamp. Normal mental status was present. Typical metachromatic granules were not seen either in bone-marrow cells or in peripheral blood cells. The X-ray picture showed spondylar and pelvic dysplasia. Qualitative rather than quantitative anomalies were shown in the urinary mucopolysaccharides, mostly involving chondroitin-6-sulfate. The genetic data are consistent with autosomal recessive inheritance. Topics: Abnormalities, Multiple; Adolescent; Adult; Child; Child, Preschool; Chondroitin; Chondroitin Sulfates; Corneal Opacity; Female; Genes, Recessive; Humans; Male; Mucopolysaccharidosis IV; Pedigree; Time Factors | 1978 |
The hand in mucopolysaccharide disorders.
Topics: Adult; Child; Child, Preschool; Chondroitin; Female; Hand; Hand Deformities, Acquired; Humans; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Occupational Therapy; Radiography; Retinitis Pigmentosa; Syndrome | 1974 |
Detection of mucopolysaccharidoses by sulphate incorporation into stimulated lymphocytes.
Topics: Chondroitin; Diagnosis, Differential; Humans; Intellectual Disability; Lectins; Lymphocytes; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Sulfates; Sulfur Radioisotopes; Syndrome; Thymidine; Tritium | 1974 |
Morquio's syndrome: deficiency of a chondroitin sulfate N-acetylhexosamine sulfate sulfatase.
Topics: Biopsy; Chondroitin; Fibroblasts; Hexosamines; Humans; Intellectual Disability; Leukodystrophy, Metachromatic; Lysosomes; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Skin; Sulfatases; Syndrome | 1974 |
Deficiency of chondroitin sulfate N-acetylgalactosamine 4-sulfate sulfatase in Maroteaux-Lamy syndrome.
Topics: Cells, Cultured; Chondroitin; Fibroblasts; Galactosamine; Humans; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Skin; Sulfatases; Sulfuric Acids; Syndrome | 1974 |
Decrease of urinary acid mucopolysaccharides in Hurler's syndrome after plasma infusion.
Topics: Blood Transfusion; Chondroitin; Corneal Opacity; Glycosaminoglycans; Humans; Mucopolysaccharidoses; Mucopolysaccharidosis I; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Sulfates; Syndrome | 1974 |
Glycosaminoglycans of iliac crest cartilage in normal children and in Morquio's disease.
Topics: Adolescent; Age Factors; Biopsy; Cartilage; Child; Child, Preschool; Chondroitin; Chromatography, DEAE-Cellulose; Female; Galactose; Glucosamine; Glycosaminoglycans; Histocytochemistry; Humans; Infant; Infant, Newborn; Lyases; Male; Mucopolysaccharidosis IV; Sulfatases | 1974 |
Glycosaminoglycans of iliac crest cartilage in spondyloepiphyseal dysplasia congenita.
Topics: Cartilage; Child; Chondroitin; Chromatography; Chromatography, DEAE-Cellulose; Disaccharides; Electrophoresis; Female; Glycosaminoglycans; Hexosamines; Humans; Ilium; Keratins; Male; Mucopolysaccharidosis IV; Uronic Acids | 1974 |
Russell bodies and plasma cells in human conjunctiva.
Topics: Biopsy; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Conjunctiva; Corneal Dystrophies, Hereditary; Corneal Opacity; Endoplasmic Reticulum; Glycosaminoglycans; Golgi Apparatus; Humans; Hyalin; Inclusion Bodies; Intellectual Disability; Macula Lutea; Microscopy, Electron; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Plasma Cells; Retinitis Pigmentosa | 1973 |
Corneal clouding in the genetic mucopolysaccharidoses: a cell culture study.
Topics: Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Chondroitin; Conjunctiva; Cornea; Corneal Opacity; Electrophoresis; Fibroblasts; Glycosaminoglycans; Histocytochemistry; Humans; Hyaluronic Acid; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Skin; Syndrome; Uronic Acids | 1973 |
Pattern of urinary acid mucopolysaccharide excretion in five patients with genetic mucopolysaccharidoses.
Topics: Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Chromatography; Corneal Opacity; Electrophoresis; Female; Galactosamine; Glycosaminoglycans; Heparitin Sulfate; Hexosamines; Humans; Hyaluronic Acid; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Sulfates; Uronic Acids | 1973 |
Identification of acid mucopolysaccharides by gas chromatographic analysis of component sugars after chemical depolymerization.
Topics: Carbohydrate Metabolism, Inborn Errors; Chondroitin; Chromatography, Gas; Chromatography, Gel; Corneal Opacity; Glycosaminoglycans; Heparin; Hexosamines; Humans; Hydrolysis; Intellectual Disability; Methanol; Monosaccharides; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Uronic Acids | 1973 |
Sequential thin layer chromatography of urinary acidic glycosaminglycans.
Topics: Carbohydrate Metabolism, Inborn Errors; Chondroitin; Chromatography, Thin Layer; Glycosaminoglycans; Heparitin Sulfate; Humans; Hyaluronic Acid; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1972 |
Simulation of genetic mucopolysaccharidoses in normal human fibroblasts by alteration of pH of the medium.
Catabolism of sulfated mucopolysaccharide by normal human fibroblasts in culture is progressively inhibited as the pH of the growth medium is raised from 6.8 to 8.0. The final cell density increases with the change in pH. The capacity to degrade mucopolysaccharide is rapidly restored by lowering the pH, and this reactivation does not require protein synthesis. Such pH dependence is not observed in cells from patients with genetic impairment of mucopolysaccharide degradation, such as the Hurler or Hunter syndromes. These results may have relevance not only to studies of mucopolysaccharide metabolism in cell culture, but also to the use of metachromasia as a genetic marker and to the observation that normal fibroblasts are released from contact inhibition of growth as the pH of the growth medium is raised. Topics: Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Chondroitin; Contact Inhibition; Corneal Opacity; Fibroblasts; Glycosaminoglycans; Humans; Hydrogen-Ion Concentration; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Skin; Sulfur Isotopes; Sulfuric Acids | 1972 |
[Mucopolysaccharidoses. I. The clinical and radiological aspects of the 6 classical mucopolysaccharidoses].
Topics: Blood Chemical Analysis; Bone and Bones; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Corneal Opacity; Deafness; Diagnosis, Differential; Dwarfism; Facial Expression; Female; Glycosaminoglycans; Heart Diseases; Humans; Infant; Intellectual Disability; Male; Mucopolysaccharidosis I; Mucopolysaccharidosis IV; Radiography; Retinitis Pigmentosa | 1972 |
[Mucopolysaccharidosis].
Topics: Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Corneal Opacity; Female; Glycosaminoglycans; Humans; Infant; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1972 |
[Mucopolysaccharidosis--synthesis and prospects].
Topics: Carbohydrate Metabolism, Inborn Errors; Chondroitin; Corneal Opacity; Glycosaminoglycans; Humans; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1972 |
Allelism, non-allelism and genetic compounds among the mucopolysaccharidoses: corrective factors in nosology, genetics and therapy.
Topics: Alleles; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Corneal Opacity; Female; Fibroblasts; Glycosaminoglycans; Homozygote; Humans; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Mutation; Phenotype; Retinitis Pigmentosa | 1972 |
Hurler's, Hunter's and Morquio's syndromes. A biochemical study in the light of current views of the underlying defects.
Glycosaminoglycans were isolated from the urine of three patients with Hurler's, Hunter's and Morquio's syndromes and also from the liver and spleen of the case of Hurler's syndrome by a procedure avoiding further degradation. A method of determining the proportions of dermatan sulphate, heparan sulphate and chondroitin sulphate in each preparation is described. The relative proportions of these glycosaminoglycans in the urine and organs of the case of Hurler's syndrome were very similar. Glycosaminoglycans from the organs were of much lower molecular weight than normal, consisting of single chains of molecular weight about 5000 together with multiples of up to four such chains attached to peptide moieties. The linkage region normally attaching glycosaminoglycan chains to protein in whole protein-polysaccharides of connective tissue was degraded progressively towards serine. The total output and relative proportions of abnormal glycosaminoglycans in the urine were compared in two brothers with Hunter's syndrome examined on two occasions 4 years apart. At comparable ages they excreted about the same amount, and the relative proportions of each glycosaminoglycan remained essentially constant. The composition and chromatographic behaviour of the glycosaminoglycan in the urine from the case of Morquio's syndrome indicated that it consisted of material containing about one-third keratan sulphate and two-thirds chondroitin sulphate as part of the same molecule, as in proteoglycans of cartilage. The total output of glycosaminoglycans, although higher than normal, was considerably less than in other types of Mucopolysaccharidoses. Topics: Amino Acids; Carbohydrates; Child; Child, Preschool; Chondroitin; Chromatography; Detergents; Glycoproteins; Glycosaminoglycans; Humans; Hyaluronoglucosaminidase; Infant; Liver; Male; Molecular Weight; Mucopolysaccharidosis I; Mucopolysaccharidosis II; Mucopolysaccharidosis IV; Pentoses; Pyridinium Compounds; Retinitis Pigmentosa; Solubility; Sulfates | 1971 |
Chondroitin 4- and 6-sulfaturia in Morquio-Ullrich's syndrome.
Topics: Adolescent; Amino Acids; Child; Child, Preschool; Chondroitin; Chromatography, Ion Exchange; Chromatography, Paper; Electrophoresis, Paper; Ethanol; Female; Galactosamine; Glucosamine; Glycosaminoglycans; Hexosamines; Hexoses; Humans; Hyaluronic Acid; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Proteus; Serine; Testis; Uronic Acids | 1971 |
Hepatic ultrastructure in chondroitin-4-sulfate mucopolysaccharidosis.
Topics: Biopsy; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Cytoplasm; Diagnosis, Differential; Female; Humans; Liver; Lysosomes; Microscopy, Electron; Mucopolysaccharidosis IV; Sulfates | 1971 |
Biochemical definition of the mucopolysaccharidoses.
Topics: Adolescent; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Chromatography; Corneal Opacity; Diagnosis, Differential; Female; Glycosaminoglycans; Heparin; Humans; Hyaluronic Acid; Infant; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1970 |
A new mucopolysaccharidosis.
Topics: Adolescent; Bone and Bones; Carbohydrate Metabolism, Inborn Errors; Child; Chondroitin; Corneal Opacity; Female; Heparin; Humans; Hypertrichosis; Intellectual Disability; Joint Diseases; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1970 |
Acid mucopolysaccharides in cultured human fibroblasts.
Topics: Acids; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Culture Techniques; Cystic Fibrosis; Fibroblasts; Gaucher Disease; Glycosaminoglycans; Humans; Hyaluronic Acid; Lipid Metabolism, Inborn Errors; Lipidoses; Marfan Syndrome; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Sulfates | 1969 |
[Heritable disorders of the connective tissue].
Topics: Adolescent; Adult; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Connective Tissue; Corneal Opacity; Ehlers-Danlos Syndrome; Female; Glycosaminoglycans; Humans; Infant; Infant, Newborn; Intellectual Disability; Male; Marfan Syndrome; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1969 |
[Diagnosis and differential diagnosis of Morquio's disease].
Topics: Adolescent; Child; Child, Preschool; Chondroitin; Diagnosis, Differential; Dwarfism; Female; Glycosaminoglycans; Humans; Mucopolysaccharidosis IV; Radiography; Sulfates | 1969 |
Urinary acid mucopolysaccharides in Morquio's disease.
Topics: Child, Preschool; Chondroitin; Glycosaminoglycans; Heparin; Humans; Male; Mucopolysaccharidosis IV; Sulfates | 1968 |
Characterization of urinary peptoglycans in Morquio's disease.
Topics: Amino Acids; Animals; Chondroitin; Chromatography; Glycosaminoglycans; Mucopolysaccharidosis IV; Peptides; Serine; Sulfates; Threonine | 1968 |
[Etiopathogenesis of mucopolysaccharidoses].
Topics: Carbohydrate Metabolism, Inborn Errors; Chondroitin; Glycosaminoglycans; Humans; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1967 |
[The mucopolysaccharidoses in childhood. Clinical study].
Topics: Carbohydrate Metabolism, Inborn Errors; Child, Preschool; Chondroitin; Dwarfism; Female; Glycosaminoglycans; Humans; Infant; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa | 1967 |
[Skin accumulation of acid mucopolysaccharides in a case of mucopolysaccharidosis with elimination of chondroitin sulfate B].
Topics: Chondroitin; Glycosaminoglycans; Histocytochemistry; Humans; Mucopolysaccharidosis IV; Skin; Sulfates | 1967 |
[Column chromatographic fractionation of acid mucopolysaccharides in the urine].
Topics: Chondroitin; Chromatography; Chromatography, Paper; Colorimetry; Diagnosis, Differential; Glycosaminoglycans; Humans; Models, Theoretical; Mucopolysaccharidoses; Mucopolysaccharidosis IV | 1966 |