TY - JOUR
T1 - Promiscuous expression of myosin in myotonic dystrophy
AU - Moore, Gudrun E.
AU - Roses, Allen D.
AU - Pericak‐vance, Margaret A.
AU - Garrett, William E.
AU - Schachat, Frederick H.
N1 - Copyright:
Copyright 2016 Elsevier B.V., All rights reserved.
PY - 1986/5
Y1 - 1986/5
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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U2 - 10.1002/mus.880090413
DO - 10.1002/mus.880090413
M3 - Article
C2 - 3713741
AN - SCOPUS:0022618882
VL - 9
SP - 355
EP - 363
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
IS - 4
ER -