The HGAR1 familial hypercholesterolemia pedigree

Data on 232 members of a single pedigree, descended from two pairs of original parents, were made available to the participants of Genetic Analysis Workshop 8 (GAW8). In addition to information concerning age and sex, measurements for 10 quantitative traits and genotypes at 22 polymorphic marker loci were also provided for a subset of 193 of these family members. © 1993 Wiley-Liss, Inc.     

Allografted keratinocytes used to accelerate the treatment of burn wounds are replaced by recipient cells

Cultured keratinocytes were used as allografts on burn wounds in two patients. In both patients successful covering of the wounds was obtained. DNA fingerprinting of the epidermis covering the wounds 21 days later showed that the cultured keratinocytes were replaced by the patients' cells.     

Primary extraosseous osteogenic sarcoma of the mediastinum: clinical, pathologic, and radiologic correlation

We have presented a rare case of primary involvement of the anterior mediastinum by osteogenic sarcoma in which CT was useful in demonstrating calcium within the mass, and in distinguishing the mass from surrounding bony structures. Although rare, extraosseous osteogenic sarcoma should be included in the differential diagnosis of a calcified anterior mediastinal mass.     

Rapid reorganization of adult rat motor cortex somatic representation patterns after motor nerve injury.

The potential for peripheral nerve injury to reorganize motor cortical representations was investigated in adult rats. Maps reflecting functional connections between the motor cortex and somatic musculature were generated with intracortical electrical stimulation techniques. Comparison of cortical somatotopic maps obtained in normal rats with maps generated from rats with a facial nerve lesion indicated that the forelimb and eye/eyelid representations expanded into the normal vibrissa area. Repeated testing from an electrode placed chronically in the motor cortex showed a shift from vibrissa to forelimb within hours after facial nerve transection. These comparatively quick changes in motor cortex representation pattern suggest that synaptic relations between motor cortex and somatic musculature are continually reshaped in adult mammals.     

Effects of physiological and pathological levels of glucocorticoids on skeletal muscle glutamine metabolism in the rat

The effects of physiological and pathological concentrations of glucocorticoids were investigated using the glucocorticoid antagonist RU486 and the synthetic glucocorticoid dexamethasone, respectively. The effects of these treatments on the concentrations of glutamine and other amino acids in skeletal muscle and plasma and on the rates of release of glutamine and alanine from incubated preparations of skeletal muscle of the rat were investigated. Dexamethasone treatment increased the concentration of glutamine and the rate of release of this amino acid from incubated soleus muscle preparations. This treatment decreased the concentration of glutamine in both gastrocnemius and EDL muscles, but was without effect on the rate of glutamine release from EDL muscles. In contrast, administration of the glucocorticoid antagonist RU486 decreased the rate of glutamine release from muscle. It is concluded that glucocorticoids have marked effects on the metabolism of glutamine by skeletal muscle per se and that these hormones may be important in the control of the rate of glutamine release from muscle in both physiological and pathological conditions.     

Phencyclidine binding to striatal cocaine receptors

PCP and related compounds inhibit 3H-mazindol binding to the cocaine receptor on dopamine transporters. The relative potencies of these compounds are such that some of the behavioral effects of PCP could be related to its action at the cocaine receptor; however, the affinity of PCP at the cocaine site (Ki = 1.59 microM) is less than its affinity at its own receptor (Ki about 0.12 microM). More data will be needed to conclusively implicate the cocaine receptor in the action of PCP.     

Free-floating thrombus of the extracranial internal carotid artery

Free-floating clots of the extracranial internal carotid artery are generally considered as surgical emergencies. This retrospective study analyzes six free-floating clots diagnosed by arteriography. Three of these patients had a fixed stroke while the other three had an evolving stroke. Three patients had antecedent ocular or hemispheric transient ischemic attacks. The causes of free-floating clots in the internal carotid artery were atheromatous stenosis in two cases, ulcerated plaque in three cases, and carotid artery dissection in one. All six patients were seen late, approximately 15 hours after their neurologic accident. They were treated with intravenous heparin over a two to five week period. Repeat arteriograms demonstrated complete clot lysis in four instances, while partial lysis was seen in one case. Moderate extension of thrombus occurred in one case only. No further neurologic complications were noted during the treatment by heparin. As indicated by follow-up arteriographic findings, secondary surgery was performed for major carotid lesions and residual clots in five cases. The free-floating thrombus syndrome of the carotid artery should not be considered as a surgical emergency when discovered late in the wake of an acute neurologic accident.     

A chemically defined medium for the culture of mature oligodendrocytes

A new chemically defined medium consisting of equal parts of Dulbecco modified Eagle's and Ham's F-12 media supplemented with insulin, sodium selenite, putrescine, and D+ galactose, which allows the long-term survival of mature oligodendrocyte pure cultures, is described. Immunohistochemical staining has shown that over 90% of the cells become positive for myelin proteins shortly following subculture. Contaminating astrocytes (2%) do not survive in this medium. Biochemical data have indicated that these purified oligodendrocytes express 2'3'-cyclic nucleotide 3'-phosphohydrolase and UDP-galactose ceramide galactosyltransferase activities. Electron microscopical examination revealed that the oligodendrocytes were mostly of medium-dark type and appeared to be identical to cells cultured in serum-containing medium. The ability to maintain pure oligodendrocyte cultures in such a defined medium will allow investigations concerning exogenous and endogenous factors involved in oligodendrocyte metabolism.