Free cytoplasmic Ca++ at rest and after cholinergic stimulus is increased in cultured muscle cells from Duchenne muscular dystrophy patients.
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 suggests that there is no generalized membrane defect.
Mongini
T
T
Clinica Neurologica II, Università degli Studi, Torino, Italy.
Ghigo
D
D
Doriguzzi
C
C
Bussolino
F
F
Pescarmona
G
G
Pollo
B
B
Schiffer
D
D
Bosia
A
A
D000109:Acetylcholine / Q000502:physiology*; D002118:Calcium / Q000378:metabolism*; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D003593:Cytoplasm / Q000378:metabolism*; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D009132:Muscles / Q000378:metabolism* / Q000473:pathology; D009136:Muscular Dystrophies / Q000378:metabolism* / Q000473:pathology; D012146:Rest; D013268:Stimulation, Chemical
D000109:Acetylcholine; D002118:Calcium