Abstract
We studied (β-spectrin using an immunologic probe in muscle samples from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), other disease controls, and normal controls. By immunohistochemistry in DMD samples, β-spectrin showed reduced or interrupted staining of the entire cell surface or only patches of bright staining at the cell periphery. There were also alterations of β-spectrin immunostain in fibers that were not degenerating or regenerating. By immunoblotting, the amount of β-spectrin in muscle was reduced and varied from 52% to 78% of the normal controls. We found normal values of β-spectrin in BMD and disease controls. These observations indicate that the expression of β-spectrin in DMD is abnormal and that β-spectrin immunolabeling is not a good marker for monitoring membrane integrity in DMD muscle.
| Original language | English |
|---|---|
| Pages (from-to) | 1149-1153 |
| Number of pages | 5 |
| Journal | Neurology |
| Volume | 44 |
| Issue number | 6 |
| Publication status | Published - 1994 |
Fingerprint
ASJC Scopus subject areas
- Arts and Humanities (miscellaneous)
- Clinical Neurology
- Neuroscience(all)
Cite this
Abnormalities in the expression of β–spectrin in duchenne muscular dystrophy. / Minetti, C.; Tanji, K.; Rippa, P. Gasparo; Morreale, G.; Cordone, C.; Bonilla, Eduardo.
In: Neurology, Vol. 44, No. 6, 1994, p. 1149-1153.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Abnormalities in the expression of β–spectrin in duchenne muscular dystrophy
AU - Minetti, C.
AU - Tanji, K.
AU - Rippa, P. Gasparo
AU - Morreale, G.
AU - Cordone, C.
AU - Bonilla, Eduardo
PY - 1994
Y1 - 1994
N2 - We studied (β-spectrin using an immunologic probe in muscle samples from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), other disease controls, and normal controls. By immunohistochemistry in DMD samples, β-spectrin showed reduced or interrupted staining of the entire cell surface or only patches of bright staining at the cell periphery. There were also alterations of β-spectrin immunostain in fibers that were not degenerating or regenerating. By immunoblotting, the amount of β-spectrin in muscle was reduced and varied from 52% to 78% of the normal controls. We found normal values of β-spectrin in BMD and disease controls. These observations indicate that the expression of β-spectrin in DMD is abnormal and that β-spectrin immunolabeling is not a good marker for monitoring membrane integrity in DMD muscle.
AB - We studied (β-spectrin using an immunologic probe in muscle samples from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), other disease controls, and normal controls. By immunohistochemistry in DMD samples, β-spectrin showed reduced or interrupted staining of the entire cell surface or only patches of bright staining at the cell periphery. There were also alterations of β-spectrin immunostain in fibers that were not degenerating or regenerating. By immunoblotting, the amount of β-spectrin in muscle was reduced and varied from 52% to 78% of the normal controls. We found normal values of β-spectrin in BMD and disease controls. These observations indicate that the expression of β-spectrin in DMD is abnormal and that β-spectrin immunolabeling is not a good marker for monitoring membrane integrity in DMD muscle.
UR - http://www.scopus.com/inward/record.url?scp=0028283357&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0028283357&partnerID=8YFLogxK
M3 - Article
C2 - 8208414
AN - SCOPUS:0028283357
VL - 44
SP - 1149
EP - 1153
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 6
ER -