Calpains and muscular dystrophies
JG Tidball, MJ Spencer - The international journal of biochemistry & cell …, 2000 - Elsevier
The international journal of biochemistry & cell biology, 2000•Elsevier
Calpains are a ubiquitous, well-conserved family of calcium-dependent, cysteine proteases.
Their function in muscle has received increased interest because of the discoveries that the
activation and concentration of the ubiquitous calpains increase in the mouse model of
Duchenne muscular dystrophy (DMD), but null mutations of muscle specific calpain causes
limb girdle muscular dystrophy 2A (LGMD2A). These findings indicate that modulation of
calpain activity contributes to muscular dystrophies by disrupting normal regulatory …
Their function in muscle has received increased interest because of the discoveries that the
activation and concentration of the ubiquitous calpains increase in the mouse model of
Duchenne muscular dystrophy (DMD), but null mutations of muscle specific calpain causes
limb girdle muscular dystrophy 2A (LGMD2A). These findings indicate that modulation of
calpain activity contributes to muscular dystrophies by disrupting normal regulatory …
Calpains are a ubiquitous, well-conserved family of calcium-dependent, cysteine proteases. Their function in muscle has received increased interest because of the discoveries that the activation and concentration of the ubiquitous calpains increase in the mouse model of Duchenne muscular dystrophy (DMD), but null mutations of muscle specific calpain causes limb girdle muscular dystrophy 2A (LGMD2A). These findings indicate that modulation of calpain activity contributes to muscular dystrophies by disrupting normal regulatory mechanisms influenced by calpains, rather than through a general, nonspecific increase in proteolysis. Thus, modulation of calpain activity or expression through pharmacological or molecular genetic approaches may provide therapies for some muscular dystrophies.
Elsevier
