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Hyaluronate synthesized by cultured skin fibroblasts derived from patients with Werner's syndrome
- Source :
- Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. 1139:84-90
- Publication Year :
- 1992
- Publisher :
- Elsevier BV, 1992.
-
Abstract
- Hyaluronate in cultured skin fibroblasts derived from patients with Werner's syndrome, who excrete large amounts of urinary hyaluronate, was investigated. The amount of hyaluronate sectreted into the medium by Werner's fibroblasts was 2–3 times that of normal fibroblasts, whereas no difference in enzyme activities related to the degradation of hyaluronate was found. Werner's fibloblasts were then cultured in the presence of [ 3 H]glucosamine, and the amount of [ 3 H]hyaluronate and its chain lengths in the medium and matrix (trypsinate) fractions were compared with those of normal cells. No significant difference in the chain lenght of hyaluronate was observed between normal and Werner's fibroblasts. On the other hand, a significant increase of hyaluronate was found in the matrix fraction of Werner's fibroblasts when the cells reached confluency. In addition, a hyaluronate of small chain length was found in thematrix fraction of Werner's fibroblasts, although this was absent from that of normal cells. It was concluded that the constituents of the extracellular matrix of Werner's fibroblasts differed from those of normal cells, characterized by the presence of a large amount of hyaluronate and a relatively small hyaluronate chain.
- Subjects :
- Adult
Male
congenital, hereditary, and neonatal diseases and abnormalities
Matrix (biology)
Extracellular matrix
chemistry.chemical_compound
Glucosamine
Humans
Hyaluronic Acid
Molecular Biology
Cells, Cultured
Chromatography, High Pressure Liquid
Skin
Werner's syndrome
chemistry.chemical_classification
Confluency
Cultured skin
Significant difference
nutritional and metabolic diseases
Fibroblasts
Middle Aged
Molecular biology
Culture Media
Extracellular Matrix
Enzyme
chemistry
Biochemistry
Molecular Medicine
Werner Syndrome
Subjects
Details
- ISSN :
- 09254439
- Volume :
- 1139
- Database :
- OpenAIRE
- Journal :
- Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
- Accession number :
- edsair.doi.dedup.....11947fdda166bebda6186cb6714b6583
- Full Text :
- https://doi.org/10.1016/0925-4439(92)90086-3