ISSN:0148-639X    

DOI:10.1002/mus.880020502

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractThe incidence of elevated serum creatine kinase (CK) and pyruvate kinase (PK) activities was compared in 20 definite carriers of Duchenne muscular dystrophy (DMD), 47 possible carriers, and 42 female controls. When adult age was not regarded as a variable, 70% of the definite carriers had elevated PK, 55% had elevated CK, and 75% had elevated PK or elevated CK or both; 38% of the possible carriers had elevated PK, 19% had elevated CK, and 40% had elevated PK or elevated CK or both. The detection efficiency of the CK test was influenced by the age of the subjects: the upper normal limit of serum CK in the adult controls was at the minimum between 21 and 35 years of age, and CK activity in some carriers declined from elevated to normal levels with increasing age. With these considerations, 70% of definite carriers had elevated CK and 80% had elevated PK and/or CK; 34% of the possible carriers had elevated CK and 43% had elevated PK and/or CK. On the basis of the PK and CK measurements, only 16 of 24 possible carrier mothers were likely to be DMD carriers, implying that the other 8 were non‐carrier mothers of new mutant son

ISSN:0148-639X    

DOI:10.1002/mus.880020503

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractA small‐scale procedure for preparing tightly coupled intact skeletal muscle mitochondria from myodystrophic (myd/myd) mice is described. Mitochondrial preparations derived from heart, liver, and skeletal muscle of myd/myd and their littermate (+/?) controls are characterized with respect to their cytochrome content and their oxidative and phosphorylative capacities. Our data indicate that there is an impairment in the NADH CoQ region of the respiratory chain of myodystrophic skeletal muscle mitochondria. Both heart and liver mitochondria of myd/myd exhibited normal activities of respiratory chain‐linked oxidative phosphorylat

ISSN:0148-639X    

DOI:10.1002/mus.880020504

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractWe report four cases of congenital muscular dystrophy; all demonstrated hypotonia and multiple contractures at birth. Strength remained stationary or improved, but the tendency for contracture formation persisted. Brief small amplitude polyphasic potentials were recorded on electromyography, and muscle biopsy revealed extensive fat and/or collagen replacement, which was out of proportion to fiber necrosis or patient strength. The consistent clinical and pathologic features of these patients and others described in the literature justify considering this disorder to be a specific nosologic entity.

ISSN:0148-639X    

DOI:10.1002/mus.880020505

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractGrafting newborn muscle is an innovative method of muscle transplant. This method overcomes hypoxia in the deeper fibers and facilitates reinnervation and revascularization of the grafted muscle fibers, thus promoting the survival and development of the characteristics of the donor muscle. The result achieved is superior to that obtained from mature muscle grafts or from minced muscle transplants. When an intact soleus from a 1‐day‐old normal mouse was grafted into a recipient soleus of a 20‐day‐old dystrophic C57BL/6J‐dy2Jmouse, the actively developing normal graft helped to improve the structure and function of the dystrophic muscle. When compared to the intact dystrophic solei, the test dystrophic muscles five to six months after operation showed increases in cross‐sectional area, in wet weight, in twitch and tetanic tension, and in the number of muscle fibers with high resting membrane potentials. This is the first procedure to have raised the muscle twitch tension in an adult dystrophic mouse to the n

ISSN:0148-639X    

DOI:10.1002/mus.880020506

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractRats were denervated in one hind limb and injected with 2,4‐dichlorophenoxyacetic acid (2,4‐D). Isotonic tetanic contractions of the muscles treated with 2,4‐D after more than 10 days of denervation revealed prolonged relaxation times similar to those of the intact side and characteristic of clinical myotonia. No myotonic discharges were observed in the muscles denervated for more than 10 days and treated with 2,4‐D. The increase in threshold for action potential generation secondary to denervation is suggested as the factor limiting the initiation of the repetitive dis

ISSN:0148-639X    

DOI:10.1002/mus.880020507

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractIn‐vitro fatty acid biosynthesis was studied in normal rat sciatic nerve and during wallerian degeneration. Normal nerve incorporated 1,3‐C14‐malonyl‐CoA and 1‐C14‐acetyl‐CoA into fatty acids by a de‐novo biosynthetic pathway. The reaction product with highest radioactivity was palmitic acid, and the free fatty acids of nerve contained 90% of the total fatty acid label. During wallerian degeneration, there was a rapid increase of fatty acid biosynthesis that reached a peak between 16 and 24 days after nerve section. Values declined to normal levels at approx

ISSN:0148-639X    

DOI:10.1002/mus.880020508

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractA patient with systemic lupus erythematosus (SLE) was treated with chloroquine therapy for four years after the onset of her illness. Nine years after cessation of chloroquine, muscle weakness developed as part of the SLE. Four muscle biopsies performed for diagnostic purposes revealed varying degrees of inflammatory change as well as distinctive cytosomes with curvilinear profiles (CCPs). These CCPs were identical to those reported in Batten disease, a degenerative disorder of children which has a clinical course different from SLE. The CCPs seen in this case of SLE are thought to result from the effect of chloroquine on membrane systems within muscle cells. This report calls attention to the fact that CCPs are not unique to Batten disease but may also occur in muscle of SLE patients treated with chloroquine.

ISSN:0148-639X    

DOI:10.1002/mus.880020509

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractShort‐duration cooling of the nerve to the extensor digitorum longus muscle of the rat in vivo induced partially reversible denervation of the muscle and atrophy in the type 2 muscle fibers. Increases in cyclic adenosine monophosphate, cyclic guanosine monophosphate phosphodiesterase, adenylate cyclase, and guanlate cyclase were observed in the denervated muscle. Treatment with gangliosides of the bovine brain cortex seemed to improve the excitability of the surviving motor units and to encourage recovery of neuromuscular trophic control, but it did not affect the nerve conduction velocity or the contractile properties of the denervated muscl

ISSN:0148-639X    

DOI:10.1002/mus.880020510

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractSerum pyruvate kinase activity was measured in several myopathic and denervating illnesses as well as in 86 potential carriers of Duchenne dystrophy. These data showed that in potential Duchenne carriers, the serum pyruvate kinase assay is more sensitive in younger patients, especially those under 20 years of age.

ISSN:0148-639X    

DOI:10.1002/mus.880020511

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY