ISSN:
1432-0533
Keywords:
Key words Calpain
;
Calpastatin
;
Muscular dystrophy
;
Neuromuscular diseases
;
Normal human muscle
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract A calcium-dependent proteinase (calpain) has been suggested to play an important role in muscle degradation in Duchenne muscular dystrophy (DMD). In immunohistochemical studies, calpain and its endogenous inhibitor (calpastatin) were located exclusively in the cytoplasm in normal human muscles. The intensity of the staining was stronger in type 1 than in type 2 fibers. Quantitative immunohistochemical study showed an increase of calpain in biopsied muscles from the patients with DMD and Becker muscular dystrophy. Abnormal increases in calpain and calpastatin were demonstrated mainly in atrophic fibers, whereas necrotic fibers showed moderate or weak immunoreactions for the enzymes. Opaque fibers and hypertrophic fibers were negative. Not all dystrophin-deficient muscle fibers necessarily showed a strong reaction for calpain. We suggest that calpain may play an important role in muscle fiber degradation, especially in the early stage of muscle degradation in muscular dystrophy.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00307642
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