chondroitin and Skin-Diseases

The roles of chondroitin sulfate (CS) and dermatan sulfate (DS) have been demonstrated in various biological events such as the construction of the extracellular matrix, tissue development, and cell signaling through interactions with extracellular matrix components, morphogens, and growth factors. Human genetic diseases, including skeletal abnormalities, connective tissue diseases, and heart defects, were reported to be caused by mutations in the genes encoding glycosyltransferases, epimerases, and sulfotransferases that are responsible for the biosynthesis of CS and DS. Glycobiological approaches revealed that mutations in CS- and DS-biosynthetic enzymes led to reductions in their enzymatic activities and in the levels of CS and DS. Furthermore, CS at the surface of tumor cells plays a key role in pulmonary metastasis. A receptor for advanced glycation end-products (RAGE) was predominantly expressed in the lung, and was identified as a functional receptor for CS chains. CS and anti-RAGE antibodies inhibited the pulmonary metastasis of not only Lewis lung carcinoma but also B16 melanoma cells. Hence, RAGE and CS are potential targets of drug discovery for pulmonary metastasis and a number of other pathological conditions involving RAGE in the pathogenetic mechanism. This review provides an overview of glycobiological studies on characterized genetic disorders caused by the impaired biosynthesis of CS, as well as DS, and on the pulmonary metastasis of Lewis lung carcinoma cells involving CS and RAGE.

Topics: Animals; Bone Diseases; Carcinoma, Lewis Lung; Chondroitin; Dermatan Sulfate; Humans; Lung Neoplasms; Mice; Receptor for Advanced Glycation End Products; Skin Diseases

Topics: Adolescent; Alkaline Phosphatase; Child; Child, Preschool; Chondrodysplasia Punctata; Chondroitin; Diagnosis, Differential; Female; Fractures, Spontaneous; Humans; Hyperostosis, Cortical, Congenital; Male; Middle Aged; Mucopolysaccharidoses; Osteitis Deformans; Osteoarthropathy, Primary Hypertrophic; Osteopetrosis; Osteosclerosis; Phosphoric Monoester Hydrolases; Radiography; Skin Diseases; Syndrome

Topics: Chondroitin; Dermatitis, Exfoliative; Glycosaminoglycans; Hair; Homocystinuria; Humans; Keratins; Methionine; Psoriasis; Skin Diseases; Sulfates; Sulfur

Dermatan sulfate and hyaluronic acid are the major glycosaminoglycan components of dermatosparactic and normal calf skin but the ratio of hyaluronic acid to dermatan sulfate is 50-70% higher in dermatosparactic calf skin than that in normal calf skin. Hyaluronic acid in normal calf skin could be extracted to 93% of the total by NaCl and guanidine hydrochloride, while in dermatosparactic calf skin 57% of hyaluronic acid remained in the insoluble material after NaCl and guanidine hydrochloride extraction. The recovery of proteodermatan sulfate in NaCl extracts was similar in normal and dermatosparactic skin. The amount of proteodermatan sulfate extracted in guanidine hydrochloride form normal calf skin was 2-4 fold higher than that from dermatosparactic calf skin. No significant difference between dermatosparactic and normal calf skin proteodermatan sulfate could be detected in terms of size of the core protein (Mr = 55,000) by sodium dodecyl sulfate polyacrylamide gel electrophoresis, amino acid analysis, immunological cross-reactivity using polyclonal antibodies against either dermatosparactic or normal calf skin core protein, tryptic peptides or size of dermatan sulfate chains (Mr = 17,000-18,000) on gel chromatography.

Topics: Animals; Cattle; Cattle Diseases; Chondroitin; Dermatan Sulfate; Glycosaminoglycans; Immunochemistry; Molecular Weight; Proteoglycans; Skin; Skin Diseases

The properties of [35S]sulfate-labeled proteoglycans secreted by normal human skin fibroblasts were compared with those synthesized by fibroblasts from three patients with Coffin-Lowry syndrome. 60-80% of secreted radioactive macromolecules from normal fibroblasts were eluted from a Sepharose CL-4B column with a mean Kav-value of 0.56 (pool 2); 3-10% of the radioactivity appeared in the exclusion volume of the column (pool 1). In contrast, 17-60% of the proteoglycans from the patients were found in the void volume. The bulk of remaining material was eluted with a mean Kav-value of 0.47. Pool 2 glycan chains from two patients exhibited an increased hydrodynamic size. Pool 1 from normal cells contained predominantly a glucuronic acid-rich proteodermatan sulfate, iduronic acid amounting for approximately 20% of glucuronic acid. In the respective proteodermatan sulfate from the patients, the relative iduronic acid content was at least 33% of that of glucuronic acid. Pool 2 material of all cell lines was characterized predominantly as iduronic acid-rich proteodermatan sulfate. In the proteoglycans from two patients the content of chondroitin 4-sulfate-derived disaccharides was increased at the expense of 6-sulfated chondroitin disaccharides. Native proteoglycans from the patients were less efficiently endocytosed by fibroblasts than their normal counterparts. Coffin-Lowry fibroblasts had a normal capability to synthesize glycosaminoglycan chains on an artificial acceptor, p-nitrophenyl-beta-D-xyloside. They were also normal in 3'-phosphoadenylylsulfate: chondroitin 4- and 6-sulfotransferase activities.

Topics: Abnormalities, Multiple; Adult; Bone and Bones; Cells, Cultured; Child, Preschool; Chondroitin; Dermatan Sulfate; Fibroblasts; Glucuronates; Glucuronic Acid; Humans; Iduronic Acid; Intellectual Disability; Male; Proteoglycans; Sex Factors; Skin; Skin Diseases; Syndrome

Changes of dermal and urinary acidic glycosaminoglycans in Lichen Sclerosus et Atrophicus (LSA) were investigated with the following results: 1. Hyaluronic acid (HA) was excreted in urine of a patient with LSA. 2. HA and low sulfate chondroitin were eluted with 0.5 M NaCl by Bio Rad AG 1- X 2 (Cl- form) column chromatography. 3. Involved dermal tissue contained only about 50% of HA in the skin of a healthy adult.

Topics: Chondroitin; Electrophoresis, Cellulose Acetate; Glycosaminoglycans; Humans; Hyaluronic Acid; Male; Middle Aged; Skin; Skin Diseases

Topics: Bone Diseases; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Connective Tissue; Gingival Hypertrophy; Glycosaminoglycans; Histocytochemistry; Humans; Infant; Infant, Newborn; Joint Diseases; Male; Methods; Skin; Skin Diseases; Skin Neoplasms; Staining and Labeling; Syndrome

Topics: Biopsy; Chondroitin; Edema; Glycosaminoglycans; Humans; Hyaluronic Acid; Inflammation; Prostaglandins; Skin; Skin Diseases; Sulfates

Topics: Adult; Blood Protein Disorders; Blood Protein Electrophoresis; Bone Marrow; Bone Marrow Cells; Brain Edema; Bronchopneumonia; Chondroitin; Culture Techniques; Fluorescent Antibody Technique; Glycoproteins; Humans; Hyaluronic Acid; Immunoelectrophoresis; Immunoglobulin G; Male; Melphalan; Myxedema; Skin; Skin Diseases; Sulfuric Acids

Topics: Aminopeptidases; Chondroitin; Collagen; Connective Tissue; Connective Tissue Cells; Enzymes; Galactosidases; Glucuronidase; Glycosaminoglycans; Humans; Hyaluronic Acid; Hydrolases; Hydroxyproline; Lysosomes; Phagocytosis; Skin Diseases

Topics: Animals; Calcification, Physiologic; Calcinosis; Calcium; Chondroitin; Depression, Chemical; Epiphyses; Glycosaminoglycans; Heparin; In Vitro Techniques; Lanthanum; Rats; Rickets; Skin; Skin Diseases; Templates, Genetic; Tibia

Topics: Animals; Biopsy; Cattle; Chondroitin; Fibroblasts; Glycosaminoglycans; Histiocytes; Humans; Hyaluronic Acid; Microscopy, Electron; Radiation Injuries; Scleroderma, Systemic; Sclerosis; Skin; Skin Diseases; Staining and Labeling

Topics: Arachnodactyly; Chondroitin; Drug Therapy; Marfan Syndrome; Pathology; Rats; Research; Skin Diseases

Topics: Chondroitin; Humans; Hyaluronic Acid; Hyaluronoglucosaminidase; Skin Diseases