Two high-affinity mAbs were prepared against Torpedo dystrophin, an electric organ protein that is closely similar to human dystrophin, the gene product of the Duchenne muscular dystrophy locus. The antibodies were used to localize dystrophin relative to acetylcholine receptors (AChR) in electric organ and in skeletal muscle, and to show identity between Torpedo dystrophin and the previously described 270/300-kD Torpedo postsynaptic protein. Dystrophin was found in both AChR-rich and AChR-poor regions of the innervated face of the electroplaque. Immunogold experiments showed that AChR and dystrophin were closely intermingled in the AChR domains. In contrast, dystrophin appeared to be absent from many or all AChR-rich domains of the rat neuromuscular junction and of AChR clusters in cultured muscle (Xenopus laevis). It was present, however, in the immediately surrounding membrane (deep regions of the junctional folds, membrane domains interdigitating with and surrounding AChR domains within clusters). These results suggest that dystrophin may have a role in organization of AChR in electric tissue. Dystrophin is not, however, an obligatory component of AChR domains in muscle and, at the neuromuscular junction, its roles may be more related to organization of the junctional folds.
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1 June 1991
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June 01 1991
Localization of dystrophin relative to acetylcholine receptor domains in electric tissue and adult and cultured skeletal muscle.
R Sealock,
R Sealock
Department of Physiology, University of North Carolina, Chapel Hill 27599.
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M H Butler,
M H Butler
Department of Physiology, University of North Carolina, Chapel Hill 27599.
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N R Kramarcy,
N R Kramarcy
Department of Physiology, University of North Carolina, Chapel Hill 27599.
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K X Gao,
K X Gao
Department of Physiology, University of North Carolina, Chapel Hill 27599.
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A A Murnane,
A A Murnane
Department of Physiology, University of North Carolina, Chapel Hill 27599.
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K Douville,
K Douville
Department of Physiology, University of North Carolina, Chapel Hill 27599.
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S C Froehner
S C Froehner
Department of Physiology, University of North Carolina, Chapel Hill 27599.
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R Sealock
Department of Physiology, University of North Carolina, Chapel Hill 27599.
M H Butler
Department of Physiology, University of North Carolina, Chapel Hill 27599.
N R Kramarcy
Department of Physiology, University of North Carolina, Chapel Hill 27599.
K X Gao
Department of Physiology, University of North Carolina, Chapel Hill 27599.
A A Murnane
Department of Physiology, University of North Carolina, Chapel Hill 27599.
K Douville
Department of Physiology, University of North Carolina, Chapel Hill 27599.
S C Froehner
Department of Physiology, University of North Carolina, Chapel Hill 27599.
Online ISSN: 1540-8140
Print ISSN: 0021-9525
J Cell Biol (1991) 113 (5): 1133–1144.
Citation
R Sealock, M H Butler, N R Kramarcy, K X Gao, A A Murnane, K Douville, S C Froehner; Localization of dystrophin relative to acetylcholine receptor domains in electric tissue and adult and cultured skeletal muscle.. J Cell Biol 1 June 1991; 113 (5): 1133–1144. doi: https://doi.org/10.1083/jcb.113.5.1133
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