A cell surface abnormality in Duchenne muscular dystrophy: Intercellular adhesiveness of skin fibroblasts from patients and carriers

1983 ◽  
Vol 63 (3) ◽  
Author(s):  
G.E. Jones ◽  
J.A. Witkowski
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Cell Surface ◽  
Skin Fibroblasts ◽  
A Cell

Duchenne muscular dystrophy: studies of cell motility in vitro

1985 ◽  
Vol 76 (1) ◽  
pp. 225-234
Author(s):  
J.A. Witkowski ◽  
V. Dubowitz
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Cell Surface ◽  
Cell Types ◽  
Muscle Fibres ◽  
A Cell ◽  
Direction Of Movement ◽  
Degenerative Disorder ◽  
Cell Surface Changes

Duchenne muscular dystrophy (DMD) is a severe degenerative disorder of skeletal muscle. It has been suggested that an abnormality of the plasma membrane may be responsible for the pathogenesis of DMD, and a number of cell surface changes have been described in DMD muscle fibres and other cell types. Alterations in cell-to-cell and cell-to-substratum adhesiveness have been reported for DMD cells and we have determined whether these alterations in cell adhesiveness affect migration of cells from DMD muscle explants. DMD cells move more rapidly and spend less time at rest than do normal or DMD carrier cells, although the differences were statistically significant only for the latter cells. An inverse relationship between cell speed and contact with surrounding cells was not observed. All cells tended to persist in their direction of movement, and there were no differences between the types of cells studied. Our results support the view that there may be a cell surface defect in DMD.

scholarly journals Cell surface abnormality in clones of skin fibroblasts from a carrier of Duchenne muscular dystrophy.

1985 ◽  
Vol 22 (2) ◽  
pp. 100-103 ◽  
Author(s):  
J Hillier ◽  
G E Jones ◽  
H E Statham ◽  
J A Witkowski ◽  
V Dubowitz
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Cell Surface ◽  
Skin Fibroblasts

scholarly journals Cytotoxicity of lectins toward skin fibroblasts from patients with Duchenne muscular dystrophy and myotonic dystrophy

1986 ◽  
Vol 31 (2) ◽  
pp. 85-91 ◽  
Author(s):  
Kousaku Ohno ◽  
Chizuko Nakano ◽  
Shougo Ishii ◽  
Kenzo Takeshita
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Myotonic Dystrophy ◽  
Skin Fibroblasts

scholarly journals Protein degradation in skin fibroblasts from patients with Duchenne muscular dystrophy

1980 ◽  
Vol 192 (1) ◽  
pp. 257-262 ◽  
Author(s):  
H E Statham ◽  
J A Witkowski ◽  
V Dubowitz
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Protein Degradation ◽  
Extracellular Space ◽  
Skin Fibroblasts ◽  
Growth State ◽  
Cell Age ◽  
Normal Controls ◽  
H 30

The rates of degradation of [3H]leucine-labelled proteins have been measured in cultures of skin fibroblasts obtained from normal controls (five subjects) and patients with Duchenne muscular dystrophy (six subjects). Cultures were incubated with [3H]leucine (10 microCi/ml) for 60 min to label “short-lived” proteins, and with [3H]leucine (5 microCi/ml) for 60 h to label “long-lived” proteins. Optimal wash procedures were devised for removal of [3H]leucine from the extracellular space and from cell pools before beginning degradation measurements. Re-utilization of [3H]leucine released from degraded labelled proteins was prevented by supplementing the medium with 4mM-leucine. Rates of degradation did not depend on the growth state of the cells or on cell age over the range used (passages eight-20). Degradation of long-lived proteins was approximately linear over a 24h period, at a rate of 1.0% per h. 30% of short-lived protein was degraded within 6h. No differences were observed between protein degradation in normal fibroblasts and in those from patients with Duchenne muscular dystrophy.

Spreading behaviour of cultured fibroblasts from carriers of Duchenne muscular dystrophy

1987 ◽  
Vol 87 (1) ◽  
pp. 163-169
Author(s):  
J.A. Pizzey ◽  
J.A. Witkowski ◽  
G.E. Jones
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Prolonged Exposure ◽  
Cell Spreading ◽  
Cellular Interaction ◽  
Cell Area ◽  
Complex Situation ◽  
Cultured Fibroblasts ◽  
Cultured Skin ◽  
A Cell

Cultured skin fibroblasts from patients with Duchenne muscular dystrophy (DMD) are more sensitive than normal cells to prolonged exposure to the ionophore monensin. In a cell spreading assay in which cells were preincubated with monensin and subsequently allowed to adhere to and spread on a glass substratum in serum-free medium for 100 min, the mean transformed cell area of normal and DMD cells was 5.97 +/− 0.11 and 5.29 +/− 0.03, respectively. Cultured fibroblasts from carriers of DMD yielded a value of 5.59 +/− 0.03, which is intermediate between, and significantly different from, the values for both normal and DMD cultures. This result would be predicted on the basis of random X-chromosome inactivation in female carriers of this disorder. However, comparison of DMD carrier cell spreading data with data obtained from pooled and summated measurements taken from separate experiments using either normal or DMD fibroblasts suggest a more complex situation. Examination of the variance of the means of cell area for the true carrier population and the summated normal and DMD population provides evidence suggesting that some form of cellular interaction may occur between the two cell genotypes in culture.

Polyacrylamide-gel-electrophoretic analysis of cultured skin fibroblasts from patients with Duchenne muscular dystrophy

1981 ◽  
Vol 9 (1) ◽  
pp. 118-119 ◽  
Author(s):  
ARTHUR H. M. BURGHES ◽  
MICHAEL J. DUNN ◽  
JAN A. WITKOWSKI ◽  
VICTOR DUBOWITZ
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Electrophoretic Analysis ◽  
Polyacrylamide Gel ◽  
Skin Fibroblasts ◽  
Cultured Skin
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