Congenital blepharophimosis, joint contractures, and muscular hypotonia.
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Biomedical subjects
Publications and source records attributed to G Karpati.
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A specific antiserum against skeletal muscle creatine phosphokinase has been obtained by immunizing chickens with purified rabbit skeletal muscle creatine phosphokinase. The antiserum was organ- rather than species-specific and reacted equally well with creatine phosphokinase from rabbit, guinea pig, and human skeletal muscle. Immunological specificity was verified by double immunodiffusion in agar gel where the precipitin line exhibited creatine phosphokinase activity. In solution the antiserum inhibited the enzymatic activity of creatine phosphokinase. By means of the immunofluorescent technique, creatine phosphokinase was localized in the intermyofibrillar space of normal human skeletal muscle fibers in cryostat sections. A higher concentration of the enzyme was found in fibers which have primarily an anaerobic enzyme profile (type II fibers) as determined by conventional histochemical reactions.
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Rats and guinea pigs were injected with 2,4-dichlorophenoxyacetate (2,4-D) in amounts sufficient to keep them myotonic for over 12 hr daily. The biceps brachii muscles were studied by histochemistry, epoxy resin histology, and electron microscopy after one to five days. Changes observed include proliferation of the longitudinal component of the sarcoplasmic reticulum, production of new myofilaments on the periphery of cells and in nuclei, activation of satellite cells, accumulation of lipid droplets, myofibrillar damage with Z-disc streaming, necrosis, and regeneration. The action mehanisms of 2,4-D are not well understood. Some of the phenomena observed could be classified as proliferative and may be analogous to the auxin action of 2,4-D in plants. The production of intranuclear filaments is probably related to the diffusion of newly formed cytoplasmic proteins into nuclei.