scholarly journals Biochemical studies on the sulphated glycosaminoglycan fraction of skin fibroblasts cultured from a patient with the Hurler syndrome

1973 ◽  
Vol 132 (3) ◽  
pp. 395-402 ◽  
Author(s):  
Ralph J. Germinario ◽  
Arthur Kahlenberg ◽  
Leonard Pinsky
Keyword(s):  
Specific Radioactivity ◽  
Cell Types ◽  
Skin Fibroblasts ◽  
Hurler Syndrome ◽  
Normal Cells ◽  
Relative Retention ◽  
Cultured Skin ◽  
Corrective Factor ◽  
Sulphated Glycosaminoglycans ◽  
Sulphated Glycosaminoglycan

1. The metabolism of the sulphated glycosaminoglycan fraction in cultured skin fibroblasts derived from a patient with the Hurler syndrome and from a normal subject was studied. Two labelled precursors, Na235SO4 and d-[2-3H]glucose, were used and their intracellular fates during uptake and `chase' periods were assessed after separation of sulphated glycosaminoglycans from hyaluronic acid. After 4 or 8h of exposure to culture medium containing both labels, [35S]sulphate incorporation into the sulphated glycosaminoglycan fraction was twofold greater in Hurler-syndrome cells than in normal cells. At the same time, the rate of incorporation of [3H]glucose into the sulphated glycosaminoglycan fraction was approximately the same for both cell types. Consequently, an increased 35S/3H ratio (nmol of [35S]sulphate incorporated/nmol of [3H]glucose incorporated) was observed for Hurler-syndrome cells compared with normal cells. 2. The results of `chase' experiments revealed that although the expected loss and relative retention of labelled sulphate occurred in the sulphated glycosaminoglycan fraction of normal and Hurler-syndrome cells, both cell types retained all of their radioactivity derived from [3H]glucose. 3. After 34h exposure to a `corrective-factor' preparation from urine, the sulphated glycosaminoglycan content (as hexosamine and [35S]sulphate) of the Hurler-syndrome cells approached normal values. At the same time, there was an increase in specific radioactivity of `corrected' Hurler-syndrome cells.

scholarly journals The disorder of hyaluronic acid metabolism in cultured skin fibroblasts derived from a patient with the Hurler syndrome

1973 ◽  
Vol 132 (3) ◽  
pp. 403-408 ◽  
Author(s):  
Ralph J. Germinario ◽  
Arthur Kahlenberg ◽  
Leonard Pinsky
Keyword(s):  
Hyaluronic Acid ◽  
Acid Content ◽  
Specific Radioactivity ◽  
Skin Fibroblasts ◽  
Hurler Syndrome ◽  
Acid Fraction ◽  
Normal Cells ◽  
Cultured Fibroblasts ◽  
Cultured Skin ◽  
Corrective Factor

The metabolism of hyaluronic acid in cultured skin fibroblasts derived from a patient with the Hurler syndrome and from a normal subject was examined. 1. An increased net incorporation of [3H]glucose into the hyaluronic acid fraction of the Hurler-syndrome cells occurred when compared with normal cells. 2. During a `chase' period, approx. 35% of the radioactivity derived from glucose was lost from the hyaluronic acid fraction of the Hurler-syndrome cells, whereas the normal cells retained all their radioactivity. 3. Although the Hurler-syndrome cells contained a ninefold greater amount of hyaluronic acid than normal cells, simultaneous determination of the specific radioactivity derived from the label revealed a value for the Hurler-syndrome cells one-half that of normal cells. These results are taken to indicate that the Hurler cells synthesize hyaluronic acid de novo at a higher rate than do normal cells. 4. Exposure of Hurler-syndrome cultured fibroblasts to a crude urine corrective-factor preparation (Neufeld & Cantz, 1971), now known to contain α-l-iduronidase, the specific Hurler-syndrome corrective factor (Bach et al., 1972), decreased the hyaluronic acid content to near-normal values before any effect was observed on [3H]glucose incorporation into the hyaluronic acid fraction. 5. In addition, the hyaluronic acid content of the normal cells decreased after exposure to the corrective factor of urine. 6. The mobilization of hyaluronic acid in Hurler-syndrome and normal cells exposed to the crude corrective-factor preparation of urine caused a decrease in specific radioactivity in the `corrected' Hurler-syndrome cells and an increase in specific radioactivity in the `corrected' normal cells.

scholarly journals Altered glycosaminoglycan production in cultured osteogenesis-imperfecta skin fibroblasts

1983 ◽  
Vol 213 (1) ◽  
pp. 171-178 ◽  
Author(s):  
H Turakainen
Keyword(s):  
Molecular Weight ◽  
Hyaluronic Acid ◽  
Osteogenesis Imperfecta ◽  
Collagen Synthesis ◽  
Gel Filtration ◽  
Exponential Phase ◽  
Skin Fibroblasts ◽  
Collagen Secretion ◽  
Sulphated Glycosaminoglycans ◽  
Sulphated Glycosaminoglycan

Collagen and glycosaminoglycan syntheses were studied in skin fibroblasts cultured from patients with osteogenesis imperfecta (OI) and from age-matched controls. Collagen synthesis (measured as protein-bound [3H]hydroxyproline) was decreased in all four OI cell lines studied in the present experiments, comprising 16-24% of total protein synthesis (40% in normal cells). Hyaluronic acid production in OI skin fibroblasts per cell was higher than in age-matched controls, but the production of sulphated glycosaminoglycans was at the normal level. Thus the ratio of the hyaluronic acid and sulphated-glycosaminoglycan radioactivities was markedly higher in OI cultures than in control cultures, especially at the exponential phase of growth where the synthesis of hyaluronic acid was highest. Hyaluronic acid in OI had a normal molecular weight when determined by gel filtration on Sepharose 2B. The removal of high-molecular-weight hyaluronic acid from the medium by hyaluronidase had no effect on the rate of collagen secretion in OI cell line 1 (A.T.C.C. 1262), in which the rate of collagen secretion was lowest.

Electron Microscopy of Fibroblasts from Myotonic Muscular Dystrophy Patients

1981 ◽  
Vol 39 ◽  
pp. 498-499
Author(s):  
S. E. Miller ◽  
G. B. Hartwig ◽  
R. A. Nielsen ◽  
A. P. Frost ◽  
A. D. Roses
Keyword(s):  
Electron Microscopy ◽  
Muscular Dystrophy ◽  
Genetic Diseases ◽  
Skin Fibroblasts ◽  
Inborn Errors ◽  
Cytoplasmic Inclusions ◽  
Cultured Skin ◽  
Myotonic Muscular Dystrophy ◽  
Glycosaminoglycan Metabolism

Many genetic diseases can be demonstrated in skin cells cultured in vitro from patients with inborn errors of metabolism. Since myotonic muscular dystrophy (MMD) affects many organs other than muscle, it seems likely that this defect also might be expressed in fibroblasts. Detection of an alteration in cultured skin fibroblasts from patients would provide a valuable tool in the study of the disease as it would present a readily accessible and controllable system for examination. Furthermore, fibroblast expression would allow diagnosis of fetal and presumptomatic cases. An unusual staining pattern of MMD cultured skin fibroblasts as seen by light microscopy, namely, an increase in alcianophilia and metachromasia, has been reported; both these techniques suggest an altered glycosaminoglycan metabolism An altered growth pattern has also been described. One reference on cultured skin fibroblasts from a different dystrophy (Duchenne Muscular Dystrophy) reports increased cytoplasmic inclusions seen by electron microscopy. Also, ultrastructural alterations have been reported in muscle and thalamus biopsies from MMD patients, but no electron microscopical data is available on MMD cultured skin fibroblasts.

scholarly journals Exosome Traceability and Cell Source Dependence on Composition and Cell-Cell Cross Talk

2021 ◽  
Vol 22 (10) ◽  
pp. 5346
Author(s):  
Rabab N. Hamzah ◽  
Karrer M. Alghazali ◽  
Alexandru S. Biris ◽  
Robert J. Griffin
Keyword(s):  
Donor Cell ◽  
Average Diameter ◽  
Cell Types ◽  
Cell Interaction ◽  
Target Cells ◽  
Remote Communication ◽  
Normal Cells ◽  
Growth Environment ◽  
Cell Components ◽  
The Impact

Exosomes are small vesicles with an average diameter of 100 nm that are produced by many, if not all, cell types. Exosome cargo includes lipids, proteins, and nucleic acids arranged specifically in the endosomes of donor cells. Exosomes can transfer the donor cell components to target cells and can affect cell signaling, proliferation, and differentiation. Important new information about exosomes’ remote communication with other cells is rapidly being accumulated. Recent data indicates that the results of this communication depend on the donor cell type and the environment of the host cell. In the field of cancer research, major questions remain, such as whether tumor cell exosomes are equally taken up by cancer cells and normal cells and whether exosomes secreted by normal cells are specifically taken up by other normal cells or also tumor cells. Furthermore, we do not know how exosome uptake is made selective, how we can trace exosome uptake selectivity, or what the most appropriate methods are to study exosome uptake and selectivity. This review will explain the effect of exosome source and the impact of the donor cell growth environment on tumor and normal cell interaction and communication. The review will also summarize the methods that have been used to label and trace exosomes to date.

scholarly journals Glycosaminoglycan synthesis by cultured skin fibroblasts from a patient with Lowe's syndrome.

1981 ◽  
Vol 256 (20) ◽  
pp. 10313-10318
Author(s):  
S. Fukui ◽  
H. Yoshida ◽  
T. Tanaka ◽  
T. Sakano ◽  
T. Usui ◽  
...  
Keyword(s):  
Skin Fibroblasts ◽  
Glycosaminoglycan Synthesis ◽  
Cultured Skin

scholarly journals Elevated Cystine Levels in Cultured Skin Fibroblasts From Patients With I-Cell Disease

1979 ◽  
Vol 13 (12) ◽  
pp. 1350-1355 ◽  
Author(s):  
Frank Tietze ◽  
Jean DeBrohun Butler
Keyword(s):  
Skin Fibroblasts ◽  
Cell Disease ◽  
Cultured Skin

Heme content of normal and porphyric cultured skin fibroblasts

1977 ◽  
Vol 15 (11-12) ◽  
pp. 1061-1070 ◽  
Author(s):  
David A. Brenner ◽  
Joseph R. Bloomer
Keyword(s):  
Skin Fibroblasts ◽  
Cultured Skin

Catalase in cultured skin fibroblasts from patients with the cerebro-hepato-renal (Zellweger) syndrome: normal maturation in peroxisome-deficient cells

1987 ◽  
Vol 923 (3) ◽  
pp. 478-482 ◽  
Author(s):  
Ronald J.A. Wanders ◽  
Anneke Strijland ◽  
Carlo W.T. van Roermund ◽  
Henk van den Bosch ◽  
Ruud B.H. Schutgens ◽  
...  
Keyword(s):  
Zellweger Syndrome ◽  
Skin Fibroblasts ◽  
Cultured Skin
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