Abstract
The pathologic changes in myotonic dystrophy (DM) skeletal muscle biopsies have been analyzed at both the histochemical and molecular level. A histochemical stain for pretyping single fibers in conjunction with sodium dodecyl sulphate-polyacrylamide gel electrophoresis allowed biochemical differences to be pinpointed in specific histochemical fiber types. These biochemical differences can be related to histochemical changes in fiber type observed in cross-section of the DM biopsies. Such changes included specific fiber type atrophy, hypertrophy, and disproportion. The pathogenesis of DM appears to be characterized by a large increase in the number of promiscuous fibers, that is, those fibers that express both fast and slow myosins. This promiscuity, which is rare in control muscle (<2%), is also prevalent at high levels in some family members at risk for DM. The observed promiscuity, although probably not a primary effect of DM, appears to be linked to the histochemical changes in fiber type observed in the DM biopsies.
| Original language | English |
|---|---|
| Pages (from-to) | 355-363 |
| Number of pages | 9 |
| Journal | Muscle and Nerve |
| Volume | 9 |
| Issue number | 4 |
| State | Published - Jul 16 1986 |
| Externally published | Yes |
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ASJC Scopus subject areas
- Clinical Neurology
- Neuroscience(all)
Cite this
Promiscuous expression of myosin in myotonic dystrophy. / Moore, G. E.; Roses, A. D.; Pericak-Vance, Margaret A; Garrett, W. E.; Schachat, F. H.
In: Muscle and Nerve, Vol. 9, No. 4, 16.07.1986, p. 355-363.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Promiscuous expression of myosin in myotonic dystrophy
AU - Moore, G. E.
AU - Roses, A. D.
AU - Pericak-Vance, Margaret A
AU - Garrett, W. E.
AU - Schachat, F. H.
PY - 1986/7/16
Y1 - 1986/7/16
N2 - The pathologic changes in myotonic dystrophy (DM) skeletal muscle biopsies have been analyzed at both the histochemical and molecular level. A histochemical stain for pretyping single fibers in conjunction with sodium dodecyl sulphate-polyacrylamide gel electrophoresis allowed biochemical differences to be pinpointed in specific histochemical fiber types. These biochemical differences can be related to histochemical changes in fiber type observed in cross-section of the DM biopsies. Such changes included specific fiber type atrophy, hypertrophy, and disproportion. The pathogenesis of DM appears to be characterized by a large increase in the number of promiscuous fibers, that is, those fibers that express both fast and slow myosins. This promiscuity, which is rare in control muscle (<2%), is also prevalent at high levels in some family members at risk for DM. The observed promiscuity, although probably not a primary effect of DM, appears to be linked to the histochemical changes in fiber type observed in the DM biopsies.
AB - The pathologic changes in myotonic dystrophy (DM) skeletal muscle biopsies have been analyzed at both the histochemical and molecular level. A histochemical stain for pretyping single fibers in conjunction with sodium dodecyl sulphate-polyacrylamide gel electrophoresis allowed biochemical differences to be pinpointed in specific histochemical fiber types. These biochemical differences can be related to histochemical changes in fiber type observed in cross-section of the DM biopsies. Such changes included specific fiber type atrophy, hypertrophy, and disproportion. The pathogenesis of DM appears to be characterized by a large increase in the number of promiscuous fibers, that is, those fibers that express both fast and slow myosins. This promiscuity, which is rare in control muscle (<2%), is also prevalent at high levels in some family members at risk for DM. The observed promiscuity, although probably not a primary effect of DM, appears to be linked to the histochemical changes in fiber type observed in the DM biopsies.
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M3 - Article
VL - 9
SP - 355
EP - 363
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
IS - 4
ER -