Abstract
Using immunocytochemical methods, the localization of dystrophin, the gene product affected in Duchenne muscular dystrophy (DMD) in aneural, differentiating human muscle cultures, was studied. Dystrophin was not demonstrable in undifferentiated myoblasts from control patients and from two patients with DMD. After myoblast fusion, the protein was found in circumscribed sarcoplasmic patches, in the perinuclear area, and along the surface of all normal multinucleate myotubes, with more mature myotubes showing predominantly sarcolemmal distribution. There was no staining in myotubes from one DMD patient and only faint diffuse fluorescence in myotubes from the second affected boy, however. These data provide further evidence that dystrophin is a sarcolemma-associated protein, that it is developmentally regulated, and that it is absent or greatly reduced in quantity in skeletal muscle cultures from patients with DMD.
| Original language | English |
|---|---|
| Pages (from-to) | 410-416 |
| Number of pages | 7 |
| Journal | American Journal of Pathology |
| Volume | 132 |
| Issue number | 3 |
| State | Published - Jan 1 1988 |
| Externally published | Yes |
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ASJC Scopus subject areas
- Pathology and Forensic Medicine
Cite this
Immunocytochemical study of dystrophin in muscle cultures from patient with Duchenne muscular dystrophy and unaffected control patients. / Miranda, A. F.; Bonilla, E.; Martucci, G.; Moraes, Carlos T; Hays, A. P.; Dimauro, S.
In: American Journal of Pathology, Vol. 132, No. 3, 01.01.1988, p. 410-416.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Immunocytochemical study of dystrophin in muscle cultures from patient with Duchenne muscular dystrophy and unaffected control patients
AU - Miranda, A. F.
AU - Bonilla, E.
AU - Martucci, G.
AU - Moraes, Carlos T
AU - Hays, A. P.
AU - Dimauro, S.
PY - 1988/1/1
Y1 - 1988/1/1
N2 - Using immunocytochemical methods, the localization of dystrophin, the gene product affected in Duchenne muscular dystrophy (DMD) in aneural, differentiating human muscle cultures, was studied. Dystrophin was not demonstrable in undifferentiated myoblasts from control patients and from two patients with DMD. After myoblast fusion, the protein was found in circumscribed sarcoplasmic patches, in the perinuclear area, and along the surface of all normal multinucleate myotubes, with more mature myotubes showing predominantly sarcolemmal distribution. There was no staining in myotubes from one DMD patient and only faint diffuse fluorescence in myotubes from the second affected boy, however. These data provide further evidence that dystrophin is a sarcolemma-associated protein, that it is developmentally regulated, and that it is absent or greatly reduced in quantity in skeletal muscle cultures from patients with DMD.
AB - Using immunocytochemical methods, the localization of dystrophin, the gene product affected in Duchenne muscular dystrophy (DMD) in aneural, differentiating human muscle cultures, was studied. Dystrophin was not demonstrable in undifferentiated myoblasts from control patients and from two patients with DMD. After myoblast fusion, the protein was found in circumscribed sarcoplasmic patches, in the perinuclear area, and along the surface of all normal multinucleate myotubes, with more mature myotubes showing predominantly sarcolemmal distribution. There was no staining in myotubes from one DMD patient and only faint diffuse fluorescence in myotubes from the second affected boy, however. These data provide further evidence that dystrophin is a sarcolemma-associated protein, that it is developmentally regulated, and that it is absent or greatly reduced in quantity in skeletal muscle cultures from patients with DMD.
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UR - http://www.scopus.com/inward/citedby.url?scp=0023783521&partnerID=8YFLogxK
M3 - Article
C2 - 3046367
AN - SCOPUS:0023783521
VL - 132
SP - 410
EP - 416
JO - American Journal of Pathology
JF - American Journal of Pathology
SN - 0002-9440
IS - 3
ER -