Inhibition of collagen expression by azelastine hydrochloride in cultured skin fibroblasts from normal individuals and scleroderma patients

1996 ◽  
Vol 288 (10) ◽  
pp. 575-578 ◽  
Author(s):  
H. Yamada ◽  
S. Tajima
Keyword(s):  
Skin Fibroblasts ◽  
Collagen Expression ◽  
Cultured Skin ◽  
Normal Individuals

Sulphamidase Activity in Leucocytes, Cultured Skin Fibroblasts and Amniotic Cells: Diagnosis of the Sanfilippo a Syndrome with the Use of a Radiolabelled Disaccharide Substrate

1981 ◽  
Vol 61 (6) ◽  
pp. 729-735 ◽  
Author(s):  
J. J. Hopwood ◽  
Helen Elliott
Keyword(s):  
Incubation Temperature ◽  
Reaction Rates ◽  
Skin Fibroblasts ◽  
Cultured Skin ◽  
Normal Individuals ◽  
Amniotic Cell ◽  
Routine Assay ◽  
Incubation Temperatures ◽  
Amniotic Cells ◽  
Normal Controls

1. Sulphamidase activity was assayed by incubation of the radiolabelled disaccharide O-(α-2-sulphamino-2-deoxy-d-glucopyranosyl)-(1 → 3)-l-[6-3H]idonic acid with homogenates of leucocytes and cultured skin fibroblasts and concentrates of urine derived from normal individuals, patients affected with sulphamidase deficiency disorder [mucopolysaccharidosis type IIIA (MPS IIIA): the Sanfilippo A syndrome], parents of such patients and patients affected with other mucopolysaccharidoses and lysosomal enzyme deficiencies. 2. the assay clearly distinguished affected homozygotes from normal controls, heterozygotes and other mucopolysaccharidoses types. 3. Sulphamidase displayed remarkable thermal stability; reaction rates were constant for at least 24 h at 60°C for leucocyte and 20 h at 37°C for cultured fibroblast preparations. Apparent Km values for fibroblast sulphamidase were 71 μmol/l at 37°C and 100 μmol/l at 50°C; the corresponding Vmax. values were 21 and 71 pmol min−1 mg−1 of protein respectively. an incubation temperature of 60h°C was used for the routine assay of sulphamidase activity in leucocytes, urine and amniotic supernatant preparations. the specific activities of fibroblast and amniotic cell sulphamidase, assessed at incubation temperatures of 37°C, were more than 10-fold the leucocyte enzyme activity at 60°C. 4. We recommend the use of radiolabelled disaccharide substrate for the assay of sulphamidase in leucocytes, skin fibroblasts and urine, for the routine enzymic detection of the sulphamidase deficiency disorder of the Sanfilippo A syndrome.

Propionate metabolism by cultured skin fibroblasts from normal individuals and patients with methylmalonicaciduria and propionicacidemia

1981 ◽  
Vol 26 (1) ◽  
pp. 28-40 ◽  
Author(s):  
Jun Oizumi ◽  
Tullio A. Giudici ◽  
Won G. Ng ◽  
Kenneth N.F. Shaw ◽  
George N. Donnell
Keyword(s):  
Skin Fibroblasts ◽  
Cultured Skin ◽  
Propionate Metabolism ◽  
Normal Individuals

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 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
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