|
|
||||||||
From Clinica Neurologica II (Drs. Mongini, Doriguzzi, Pollo, and Schiffer) and Dipartimento di Genetica (Sezione di Chimica Medica) (Drs. Ghigo, Bussolino, Pescarmona, and Bosia), Università degli Studi, Torino, Italy.
We used a fluorescent dye, quin 2, to measure intracellular free calcium ([Ca+ +]i) in cultured skeletal muscle cells and skin fibroblasts from five Duchenne muscular dystrophy (DMD) patients and from five controls. We observed an enhanced [Ca+ +]i level, at rest and after acetylcholine (ACh) stimulation, in DMD muscle cells, but we did not detect any difference between DMD and normal skin fibroblasts. The abnormally higher [Ca+ +]i transient induced by ACh suggests that it plays a critical role in muscle degeneration. The skin fibroblast results suggest that there is no generalized membrane defect.
Address correspondence and reprint requests to Dr. Mongini, Clinica Neurologies II, Via Cherasco 15, 10126, Torino, Italy.
Presented in part at the thirty-ninth annual meeting of the American Academy of Neurology, New York, NY, April 1987.
Received April 24, 1987. Accepted for publication in final form July 28, 1987.
This article has been cited by other articles:
![]() |
M. J. Spencer and R. L. Mellgren Overexpression of a calpastatin transgene in mdx muscle reduces dystrophic pathology Hum. Mol. Genet., October 2, 2002; 11(21): 2645 - 2655. [Abstract] [Full Text] [PDF] |
||||
![]() |
D. J. Blake, A. Weir, S. E. Newey, and K. E. Davies Function and Genetics of Dystrophin and Dystrophin-Related Proteins in Muscle Physiol Rev, April 1, 2002; 82(2): 291 - 329. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. W. Berchtold, H. Brinkmeier, and M. Muntener Calcium Ion in Skeletal Muscle: Its Crucial Role for Muscle Function, Plasticity, and Disease Physiol Rev, July 1, 2000; 80(3): 1215 - 1265. [Abstract] [Full Text] [PDF] |
||||
![]() |
J. M. Alderton and R. A. Steinhardt Calcium Influx through Calcium Leak Channels Is Responsible for the Elevated Levels of Calcium-dependent Proteolysis in Dystrophic Myotubes J. Biol. Chem., March 24, 2000; 275(13): 9452 - 9460. [Abstract] [Full Text] [PDF] |
||||
![]() |
F. W. Hopf, P. Reddy, J. Hong, and R. A. Steinhardt A Capacitative Calcium Current in Cultured Skeletal Muscle Cells Is Mediated by the Calcium-specific Leak Channel and Inhibited by Dihydropyridine Compounds J. Biol. Chem., September 13, 1996; 271(37): 22358 - 22367. [Abstract] [Full Text] [PDF] |
||||
![]() |
J. P. Earnest, G. F. Santos, S. Zuerbig, and J. E. B. Fox Dystrophin-related Protein in the Platelet Membrane Skeleton J. Biol. Chem., November 10, 1995; 270(45): 27259 - 27265. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. J. Spencer, D. E. Croall, and J. G. Tidball Calpains Are Activated in Necrotic Fibers from mdx Dystrophic Mice J. Biol. Chem., May 5, 1995; 270(18): 10909 - 10914. [Abstract] [Full Text] [PDF] |
||||
![]() |
P. Fong, P. Turner, W. Denetclaw, and R. Steinhardt Increased activity of calcium leak channels in myotubes of Duchenne human and mdx mouse origin Science, November 2, 1990; 250(4981): 673 - 676. [Abstract] [PDF] |
||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |