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contracture/protease

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Calpainopathy: Description of a Novel Mutation and Clinical Presentation with Early Severe Contractures.

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Presented here are five members of a family that was ascertained from an isolated, consanguineous, indigenous Amerindian community in Colombia that was affected with calpain 3-related, limb-girdle muscular dystrophy type R1. These patients are homozygous for a unique and novel deletion of four bases

Effects of gabexate, a protease inhibitor, on smooth muscle of guinea-pig stomach fundus.

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A protease inhibitor, gabexate (ethyl-p-6-guanidinohexanoyloxy benzoate), was found to have an antimuscarinic action in muscle strips of the guinea-pig gastric fundus. Gabexate reversibly inhibited carbachol-induced contractions in the presence of prostaglandin synthesis inhibitors (indomethacin or

The effect of muscle extracts on the contracture response of skeletal muscle to acetylcholine.

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Preincubation of normal rat soleus muscles in vitro with homogenates prepared from mixed leg muscles which had been denervated 4 days previously resulted in an increase in the contracture response to acetylcholine. After 30 min incubation a 1.5-fold increase was observed. Homogenates of normally

Role of protease inhibition in myocardial preservation following ischemia and reperfusion.

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Aprotinin, a naturally occurring protease inhibitor, in concentrations of 10(6) KIU/L was found to have no effect on myocardial performance in normally perfused isolated rat hearts, before ischemia. Given during the preischemic period, the drug had a significant protective effect on the reperfused

Treatment of mouse muscular dystrophy with the protease inhibitor pepstatin.

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Dystrophic mice were treated for 5 weeks beginning at 3 weeks of age with 20 ugm per day of pepstatin, a potent inhibitor of cathepsin D. Mortality was less and weight gain greater in pepstatin treated mice than in controls. Muscle bulk was greater and hind lamb contractures were reduced in treated

[Effect of proteases on sugar transport in muscle tissue].

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Effects of trypsin and pronase on D-xylose uptake were studied on isolated frog sartorius muscle. Trypsin and pronase exerted insulin-like effects on the transport of sugar. The acceleration of xylose transport by insulin was reduced by a prior incubation of muscles with trypsin or pronase. The

Activation of proteases and changes in Na+-K+-ATPase subunits in hearts subjected to ischemia-reperfusion.

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Previous studies have shown that ischemia-reperfusion (I/R) injury is associated with cardiac dysfunction and changes in sarcolemmal Na(+)-K(+)-ATPase subunits and activity. This study was undertaken to evaluate the role of proteases in these alterations by subjecting rat hearts to different times
Potassium pyroantimonate histochemistry, coupled with ethyleneglycoltetraacetic acid (EGTA)-chelation and X-ray microprobe analysis, was employed to localize intracellular calcium binding sites in the normal and degenerating flight musculature in queens of Solenopsis, the fire ant. In normal

Clinical and pathological features in 15 Chinese patients with calpainopathy.

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BACKGROUND Calpainopathy is comprised of a group of myopathies caused by deficiency in calcium-activated, neutral protease (calpain-3). In this study we identify calpainopathy in a cohort of Chinese patients with unclassified myopathy and analyze its clinical and pathological

Mechano-sensitive linkage in excitation-contraction coupling in frog skeletal muscle.

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1. Single skeletal muscle fibres of Xenopus laevis were used to investigate the involvement of a mechano-sensitive link in excitation-contraction coupling (EC coupling). 2. Fibres were stimulated by intermittent tetani until tension fell to about 40% of its initial level. Fibres were then stressed

Release of hydrolases and acetylcholine sensitivity in rat skeletal muscle.

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Cytosol prepared from the hind-limb muscles of the rat contained factors which increased the acetylcholine (ACh) contractures in intact normally innervated muscles in vitro. Muscles which had been denervated 4-6 days previously and normally innervated muscles released a factor upon incubation which

A peptide inhibitor of c-Jun NH2-terminal kinase reduces myocardial ischemia-reperfusion injury and infarct size in vivo.

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The c-Jun NH(2)-terminal kinase (JNK) pathway of the mitogen-activated protein kinase (MAPK) signaling cascade regulates cell function and survival after stress stimulation. Equally robust studies reported dichotomous results suggesting both protective and detrimental effects of JNK during

Biological activities of Leptodeira annulata (banded cat-eyed snake) venom on vertebrate neuromuscular preparations.

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The physiological properties of colubrid snake venoms are largely unknown and less frequently investigated. In this study, we assessed the enzymatic properties and biological activities of Leptodeira annulata (banded cat-eyed snake) venom, an opistoglyphous snake from Colombia. The proteolytic,

Atypical nemaline myopathy with temporomandibular ankylosis.

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A young girl who initially presented with jaw hypomobility at age 4 years subsequently developed signs of a slowly progressive myopathy. Rods were found in all sampled muscles, and were associated with fibrous contractures in the temporomandibular joint muscles. Rods in fibers undergoing necrosis
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