摘要:

AbstractCultured human skin fibroblasts from 9 patients with Duchenne muscular dystrophy (DMD) and 8 normal age‐ and sex‐matched controls were examined for creatine kinase (CK) activity. Both the normal and the DMD fibroblasts were found to have significant levels of CK activity (approximately 10 × 10−3IU per milligram of fibroblast protein). The control cells had slightly higher CK activity than the DMD lines, but this difference was not significant (0.2

ISSN:0148-639X    

DOI:10.1002/mus.880050102

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

年代:1982

数据来源: WILEY

摘要:

AbstractThe extensor of the second digit of the chicken wing (EDII) is a small, fast‐twitch muscle susceptible to chicken muscular dystrophy and well‐suited for correlated studies of the morphology and physiology of identified nerve and muscle fibers. In cross section, the dystrophic EDII show morphological abnormalities common to other well‐studied dystrophic chicken muscles. However, in contrast to other dystrophic chicken muscles, living endplates can be viewed in the EDII, facilitating electrophysiological studies. A survey of electrophysiological properties of the EDII muscle in birds 5–16 weeks ex ovo revealed that compared to normal preparations, the dystrophic preparations had: (1) lower resting potentials, (2) lower miniature endplate potential frequency, (3) abortive nerve‐evoked action potentials recorded extrajunctionally, and (4) multiple muscle fiber action potentials to a single depolarizing curr

ISSN:0148-639X    

DOI:10.1002/mus.880050103

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

年代:1982

数据来源: WILEY

摘要:

AbstractThe protonmotive force (Δp) of muscle mitochondria was measured by estimating the distribution of14C‐labeled TPMP (trimethylphenylphosphonium iodide) and14C‐labeled acetate across the inner membrane of muscle mitochondria. The matrix volume was simultaneously determined using3H‐labeled H2O and3H‐labeled mannitol and repeated drying to distinguish the label in these 2 compounds. Rapid separation of mitochondria from the incubation medium by centrifugation through silicone oil avoids the problems of potential anaerobic conditions associated with conventional centrifugation and large volumes of trapped media associated with filtration. The value for Δp (mean ± SD) was 192 ± 26 mV in 30 determinations with rat muscle mitochondria during state 4. Measurement of oxygen consumption allowed calculation of membrane conductance (Cm, H+) which was 0.49 ± 0.18 nmol of H+/min/mg protein/mV. The values for δp and Cm, H+are reported for a variety of experimental conditions and are consistent with Mitchell's chemiosmotic theory. Biopsy specimens obtained from human muscle gave state‐4 Δp values of 197 ± 30 mV (n= 5) and Cm, H+values of 0.52 ± 0.12 nmol of H+/min/mg/mV (n= 4). This Δp assay is the first described for coupled mammalian muscle mitochondria and will be useful in assessin

ISSN:0148-639X    

DOI:10.1002/mus.880050104

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

年代:1982

数据来源: WILEY

摘要:

AbstractThe Syrian hamster polymyopathy is a hereditary disease, transmitted by an autosomal recessive gene, involving the heart and the entire musculature. The chronology of the pathologic events in the myocardium and skeletal muscle has been investigated in UM‐X7.1 myopathic hamsters aged 0–250 days. A phasic pattern in the progression of the disease process was evident. Microscopic necrotic changes in the heart were visible prior to or at 50 days of age with increasing severity until 100 days of age and subsidence thereafter. More than 50% of the animals died before 250 days of age with signs of cardiac failure. The intensity and extent of myocardial calcific changes together with scar formation were determinant factors in curtailing the survival of animals. Changes in serum creatine kinase (CK) activity followed a phasic pattern similar to the progression of the myopathic disease. Because of the disparity of disease manifestations between the different myopathic hamster lines, it is essential to consider the time course of the heart and skeletal muscle microscopic changes when evaluating the severity of the hamster polymyopa

ISSN:0148-639X    

DOI:10.1002/mus.880050105

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

年代:1982

数据来源: WILEY

摘要:

AbstractThe mitochondrial oxidative phosphorylation, calcium and magnesium contents, and swelling‐contraction activity were investigated in relation to the progression of the hereditary hamster cardiomyopathy. The assessment was made in animals between 22 and 232 days of age, which were divided into 7 groups according to stage of disease. In 24‐day‐old hamsters prior to development of heart necrotic changes, the membrane permeability of isolated mitochondria was altered. In 50‐day‐old animals, at a stage of disease when myocardical cells undergo degeneration, a defect of oxidative phosphorylation resulting from an increase in mitochondrial calcium was demonstrated. With culmination of the heart necrotic changes, at close to 100 days of age, mitochondrial dysfunction and calcium overload were maximal. There was a transient improvement during the healing stage, but the situation deteriorated with the occurrence of circulatory failure. Since the mitochondrial respiratory pattern and calcium overload parallel the cardiac degeneration, it is inferred that the cell energy depletion is a functional consequence of an abnormal calci

ISSN:0148-639X    

DOI:10.1002/mus.880050106

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

年代:1982

数据来源: WILEY

摘要:

AbstractGanglioside‐ and glycoprotein‐bound sialic acid in endplate and non‐endplate regions of rat diaphragm muscles were assessed 1–16 days after phrenic nerve transection. Sialic acid in glycoproteins (90% of total) and gangliosides (10% of total) from intact hemidiaphragms was distributed uniformly throughout endplate and nonendplate regions. After denervation, the total sialic acid per hemidiaphragm increased (days 1–4) and reached a maximum level (days 8–12) which remained constant (days 12–16). These sialic acid changes reflected increments in gangliosides (40% over control), which occurred simultaneously in endplate and nonendplate regions (days 1–8), and increments in sialoglycoproteins (70%–80% over control), which took place much earlier in endplate (days 1–8) than in nonendplate (days 8–14) regions. The results of this study, together with previous data, favor the hypothesis that maintenance of both sialoglycoproteins and gangliosides depends upon an intact innervation, probably through separate mechanisms involving differe

ISSN:0148-639X    

DOI:10.1002/mus.880050107

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

年代:1982

数据来源: WILEY

摘要:

AbstractUsing the immunoperoxidase technique, antibodies to the fast components of the troponin complex stained all regenerating cells after localized alcohol injury to rat skeletal muscle. Antibodies to slow troponin components stained only some of these cells. About 6 weeks after injury with the nerve intact, the fast and slow forms of the troponin components were located in different cells. During the later stages of regeneration, staining for myosin ATPase correlated with the staining with antibodies to fast and slow troponin components. A similar staining pattern was also observed in the early stages of regeneration of muscle denervated at the time of injury. In this case, antibodies to fast skeletal muscle troponin components continued to stain all the cells 10 weeks after injury. Injured denervated muscle cells stained equally dark by myosin ATPase after preincubation at pH 9.4 over this period. None of the regenerating myotubes in denervated muscle stained for myosin ATPase after preincubation at pH 4.3.

ISSN:0148-639X    

DOI:10.1002/mus.880050108

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

年代:1982

数据来源: WILEY

摘要:

AbstractSera from patients with amyotrophic lateral sclerosis (ALS) were assayed for a neuron‐specific cytotoxic effect on long‐term organized cultures of neonatal mouse anterior horn segments. Blind studies show that ALS sera when incorporated into the culture media have a greater degree of antineuronal toxicity than sera from patients with other neurological diseases or from family members of ALS patie

ISSN:0148-639X    

DOI:10.1002/mus.880050109

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

年代:1982

数据来源: WILEY

摘要:

AbstractPolyglactin 910, a resorbable synthetic material, was used as a mesh‐tube to bridge defects (7 to 9 mm in length) in sectioned rabbit tibial nerve. After absorption of the mesh a new nerve sheath was formed which enclosed numerous minute fascicles of regenerating axons. The polyglactin tube influenced the direction taken by the regenerating axons and guided them into the distal segment. The tube also reduced the formation of neuromas and the growth of scar tissue from surrounding structure

ISSN:0148-639X    

DOI:10.1002/mus.880050110

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

年代:1982

数据来源: WILEY

摘要:

AbstractThe usefulness of serum creatine kinase (CK) activity in the detection of Duchenne muscular dystrophy (DMD) carriers is dependent upon a reliable control distribution. In controls there is a small but reproducible seasonal variation in CK activity, with a statistically significant variation in the upper 95th percentile (78 IU/liter in May, 53 IU/liter in November, as compared with 67 IU/liter for the whole calendar year). Because CK values in DMD heterozygotes are higher in November than in May, the carrier detection rate may be highest in November. Failure to consider this seasonal variation in controls may cause misclassification of too many normal subjects. If tests are conducted throughout the year, however, the seasonal influence can be reduced by serial testing at intervals of several months. In this case, use of the highest of the results obtained in 3 tests compared with the normal range of single measurements misclassifies too many normal subjects. Use of the mean of 3 determinations provides more accurate classification.

ISSN:0148-639X    

DOI:10.1002/mus.880050111

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

年代:1982

数据来源: WILEY