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Biomedical subjects

V Schurig

Publications and source records attributed to V Schurig.

66 records · Page 4Linked to original sources

Cytochrome P-450-catalyzed asymmetric epoxidation of simple prochiral and chiral aliphatic alkenes: species dependence and effect of enzyme induction on enantioselective oxirane formation.

The enantioselectivity of the in vitro conversion of simple prochiral and chiral aliphatic alkenes into oxiranes by liver microsomes of untreated or induced (phenobarbital) rats, of untreated or induced (phenobarbital, benzo[a] pyrene) mice, and of humans was determined by complexation gas chromatography. The enantiomeric excess (ee) of the epoxides extends from 0 (trimethyloxirane) to 50% (ethyloxirane). The configuration (R or S) of the enantiomers formed in excess is consistent for homologous oxiranes but is species dependent and in some cases influenced by enzyme induction. Enantioselectivity differences of aliphatic alkene epoxidation by human liver microsomes of four individuals are negligible.

Alkenes↗

[Epidermolysis bullosa hereditaria dystrophica (Hallopeau-Siemens). Case report and therapy trial with (+)-cyanidanol-3 in 3 patients].

The diagnosis of hereditary epidermolysis bullosa dystrophica (Hallopeau-Siemens) in three patients was confirmed by clinical and histological means as well as by electron microscopy. The clinical course was followed for many years. Since (+)-cyanidanol-3 has been shown to reduce the susceptibility of collagen to mammalian collagenase under in vitro conditions, (+)-cyanidanol-3 was used to treat the three patients for a period of 27 weeks. A decrease in the amount of blistering was observed in all three patients.

Adult↗

[Hereditary bullous epidermolyses. Recent aspects of diagnosis and therapy].

The introduction of antibodies specific for distinct basement membrane zone components is helpful in the diagnosis of hereditary mechanobullous diseases. In addition, these reagents have implications for investigation of the pathogenesis of these disorders, which might help to establish the classification based on molecular defects. Antibodies by means of indirect immune fluorescent microscopy can play a key role in the differential diagnosis of junctional and dystrophic types of epidermolysis bullosa. In epidermolysis bullosa hereditaria dystrophica, a disturbance in the regulation of collagenase activity has already been well documented, and several therapeutic approaches have been carried out based on the inhibition of collagenase activity. Concomitant inhibition of collagenolytic activity and stabilization of collagen fibrils might furthermore be helpful in the treatment of these patients. There are already several reports on the therapeutic effects of collagenase inhibitors, and stabilization of collagen fibrils in vitro has been demonstrated by (+)-cyanidanol-3. The preliminary results on therapeutic application of this drug seem to be promising.

Antibodies, Monoclonal↗

[Ichthyosis hystrix with parakeratosis in the form of cornoid lamellae].

We report on a keratinization disorder in four brothers in a family in which members had been affected in three generations. Clinical signs and genetic, histopathologic, autoradiographic and ultrastructural examinations all support the diagnosis of hystrixlike, proliferative ichthyosis with cornoid lamellae and autosomal dominant inheritance. The relationship of the disorder to other hystrixlike genetic keratinization disorders is discussed.

Adolescent↗

Biological activation of 1,3-butadiene to vinyl oxirane by rat liver microsomes and expiration of the reactive metabolite by exposed rats.

When 1,3-butadiene is incubated with rat liver microsomes and NADPH both enantiomers of vinyl oxirane are formed, the amount of epoxide being dependent on incubation time, microsomal protein, and substrate concentration. Inhibition by SKF 525 A or dithiocarb as well as induction by pretreatment with phenobarbital or 20-methylcholanthrene suggest participation of cytochrome P-450 in this reaction. The amount of epoxide is enhanced by addition of 1,1,1-trichloropropene oxide and reduced by glutathione, especially in the presence of hepatic cytosol. When rats are exposed to 1,3-butadiene in a closed chamber (conditions of maximal metabolism) vinyl oxirane is exhaled and can be quantitatively determined from the gas phase. The concurrent exhalation of acetone is consistent with the idea of biologic action of a reactive metabolite.

Acetone↗

[Hereditary angioneurotic edema. Clinical syndrome and therapy].

Hereditary angioneurotic edema is transmitted as an autosomal dominant disease. It is characterized by a deficiency of the C1 esterase inhibitor. Clinical manifestations are periodic swelling of the extremities, face, upper airways and abdominal viscera. We described one patient to demonstrate the clinical features and current treatment. Danazol therapy is discussed.

Adult↗

Nonprogressive cerebellar disorder with mental retardation and autosomal recessive inheritance in Hutterites.

We report a nonprogressive neurological disorder in at least 11 Hutterites with healthy but consanguineous parents. In several of the affected, hypotonia was noted at birth. Retarded motor and mental development became apparent during the first year of life. The age of unsupported walking varied from 5-21 years. Consistent signs were unsteady, broadly based gait and stance, exaggerated deep tendon reflexes mainly in the lower limbs, and mild to moderate mental retardation. Variable signs were extensor plantar reflexes (9/11), short stature (-2SD in 8/11), strabismus (7/11), small muscle mass (6/11), mild intention tremor (3/11), cataracts (1/11), and epilepsy (1/11). CAT scans in two affected sisters showed slight enlargement of the fourth ventricle in one and hypoplasia of the cerebellum in both. The disorder is probably the same as that described earlier under the heading, dysequilibrium syndrome.

Adolescent↗

The Meckel syndrome in the Hutterites.

At least three cases of the Meckel syndrome have been identified in the Hutterites. Two of these were sibs and were studied during life; the other, a close relative, was diagnosed retrospectively by a review of hospital records. All parents were consanguineous. The phenotype ranged in severity from the association of occipital meningocele, cystic kidneys, postaxial polydactyly, and microphthalmia to a milder expression consisting of cystic kidneys, ocular defects apparent only on funduscopic examination, and a brain abnormality demonstrated by computer tomography. Survival ranged from 5 to 13 months. In one patient, the renal lesion was manifested as a tubular rather than a glomerular defect, and was probably not the primary cause of death.

Canada↗

Determination of the enantiomerization barrier of chlorthalidone by dynamic electrokinetic chromatography and computer simulation.

Dynamic electrokinetic chromatography (DEKC) and computer simulation have been used to determine the enantiomerization barrier of the drug chlorthalidone, which is commonly employed as a diuretic. The separation of enantiomers was performed in an aqueous 100 mM citric acid/phosphate buffer system at pH 5 containing 12.5 mg/mL carboxymethyl-beta-cyclodextrin and dimethyl-beta-cyclodextrin each as a dual chiral recognition system. The enantiomerization that occurred during separation resulted in a peak distortion, i.e., plateau formation and peak broadening. The interconversion profiles were simulated with ChromWin software, which is based on the theoretical plate as well as on the stochastic model using the experimental data plateau height, hplateau; peak width at half height, Wh, and total retention time, tR. Peak form analysis yielded the rate constants, k1 and k(-1) as well as the kinetic activation parameters, deltaG++, deltaH++, and deltaS++ of the enantiomerization reaction of chlorthalidone 1. At 37.5 degrees C, the enantiomerization barrier, deltaG++, was determined to be 100.6 +/- 0.4 kJ/mol(-1); the activation parameters by temperature-dependent measurement, deltaH++ = 69.2 +/- 0.2 kJ/mol , deltaS++ = -101 +/- 6 J K(-1) mol(-1) and the half-life, tau, was found to be approximately 2.5 hr.

Chemical Phenomena↗