Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria.

Defects of complex I of the mitochondrial respiratory chain are important causes of neurological disease. We report studies that demonstrate a severe deficiency of complex I activity with less severe abnormalities of complexes III and IV (less than 5, 63, and 30% of control values, respectively) in a skeletal muscle mitochondrial fraction from a 22-yr-old female with weakness, lactic acidemia, and the deposition of intramuscular neutral lipid. The observation that lipid accumulates in this and other patients with complex I deficiency suggests impaired mitochondrial fatty acid oxidation.

Tandem direct duplications of mitochondrial DNA in mitochondrial myopathy: analysis of nucleotide sequence and tissue distribution.

Two patients with direct tandem duplications of mitochondrial DNA (mtDNA) and mitochondrial myopathy are described. The breakpoint regions between duplicated segments were amplified using the polymerase chain reaction (PCR), cloned and sequenced. The distribution of normal and abnormal genomes in different tissues was investigated using Southern hybridisation, and in different cells within the same tissue using PCR.

Effect of Castanospermine and Related Polyhydroxyalkaloids on Purified Myrosinase from Lepidium sativum Seedlings.

Myrosinase (beta-thioglucoside glucohydrolase, EC 3.2.3.

Optimism, neuroticism, coping, and symptom reports: an alternative interpretation of the Life Orientation Test.

Found in two studies that the Life Orientation Test (LOT) had limited discriminant validity relative to measures of neuroticism. Furthermore, although previous correlations of the LOT with measures of symptom reports and coping behaviors were replicated, these correlations were eliminated when neuroticism was controlled. In contrast, the correlations of symptoms and coping with neuroticism remained significant when LOT scores were controlled.

Duplications of mitochondrial DNA in mitochondrial myopathy.

Restriction enzyme analysis was done on total cellular DNA extracted from whole blood in two patients with mitochondrial myopathy and multisystem involvement and their families. The two patients had an abnormal mitochondrial genome with a large (about 8 kb) duplication present in several tissues. Normal mitochondrial DNA (mtDNA) was also present, but within each maternal lineage the abnormal mitochondrial genome was confined to clinically affected individuals.

Restriction enzyme analysis of the mitochondrial genome in mitochondrial myopathy.

The mitochondrial myopathies are a heterogeneous group of disorders some of which may be caused by mutations in the mitochondrial genome. Mitochondrial DNA from 10 patients with mitochondrial myopathy and their mothers was analysed using five restriction enzymes and 11 mitochondrial probes in bacteriophage M13. No abnormalities were found in seven out of the 10 patients.

Serological survey for rabies antibodies in raptors from California.

Fifty-three newly captive birds of prey were tested serologically for neutralizing antibodies against rabies virus, using a fluorescent focus inhibition test. No significant antibody titers were detected with this sensitive and specific technique in any of these birds. This study supports the contention that free-ranging birds of prey are of limited importance in the epidemiology of rabies.

Catabolism of Cyanogenic Glycosides by Purified Vicianin Hydrolase from Squirrel's Foot Fern (Davallia Trichomanoides Blume).

Vicianin hydrolase, which catalyzes the hydrolysis of vicianin (K(m), 4.9 millimolar) to (R)-mandelonitrile and vicianose at an optimum pH of 5.5, was extensively purified from the young fronds and fiddleheads of the squirrel's foot fern (Davallia trichomanoides Blume) using DEAE-cellulose and Ultrogel HA chromatography.

Glucosinolate Biosynthesis: Sulfation of Desulfobenzylglucosinolate by Cell-Free Extracts of Cress (Lepidium sativum L.) Seedlings.

After removal of myrosinase activity by concanavalin A-Sepharose 4B chromatography, cell-free extracts of light-grown cress (Lepidium sativum L.) seedlings, catalyzed the sulfation of desulfobenzylglucosinolate (K(m), 0.23 millimolar) to benzylglucosinolate using PAPS (K(m), 1 millimolar) as sulfur donor.

Localization and catabolism of cyanogenic glycosides.

The catabolism of cyanogenic glycosides is initiated by cleavage of the carbohydrate moiety by one or more beta-glycosidases, which yields the corresponding alpha-hydroxynitrile. Until recently, the mode by which cyanogenic disaccharides are hydrolysed was largely unclear. Investigation of highly purified beta-glycosidases from plants containing cyanogenic disaccharides has now indicated that these compounds may be degraded via two distinct pathways, depending on the plant species.

Diagnosis of rotavirus gastroenteritis by smell.

Clinical features cannot differentiate rotavirus gastroenteritis from other types of diarrhoea. Sixty eight stool specimens were examined by nurses on an infant gastroenteritis ward. Of these, 69% were correctly classified by smell alone.

Isolation and characterization of multiple forms of prunasin hydrolase from black cherry (Prunus serotina Ehrh.) seeds.

Three forms of prunasin hydrolase (PH I, PH IIa, and PH IIb), which catalyze the hydrolysis of (R)-prunasin to mandelonitrile and D-glucose, have been purified from homogenates of mature black cherry (Prunus serotina Ehrh.) seeds. Hydroxyapatite chromatography completely resolved PH I from PH IIa and PH IIb.

Dilated cardiomyopathy associated with haemolytic uraemic syndrome.

In two children with the haemolytic uraemic syndrome dilated cardiomyopathy occurred in the absence of hypertension, or fluid or electrolyte disturbance. These cases presented with acute left ventricular failure. Echocardiography showed left ventricular dilatation and reduced contractility.

Medical teaching of the cultural aspects of ethnic minorities: does it exist?

The development of published material relating to the practice of medicine in multiracial and multicultural Britain is briefly reviewed. The utilization of such information in English medical schools is found to be absent or at a low level of priority. A more detailed study of one region demonstrates that junior hospital doctors believe from experience that they have a need for training in 'multicultural' medicine to serve their current patient load.

Isolation and characterization of multiple forms of mandelonitrile lyase from mature black cherry (Prunus serotina Ehrh.) seeds.

Five multiple forms (forms 1-5) of mandelonitrile lyase (EC 4.1.2.

Comparison of kinetic and molecular properties of two forms of amygdalin hydrolase from black cherry (Prunus serotina Ehrh.) seeds.

Two forms of the beta-glucosidase amygdalin hydrolase (AH I and II), which catalyze the hydrolysis of (R)-amygdalin to (R)-prunasin and D-glucose, have been purified over 200-fold from mature black cherry (Prunus serotina Ehrh.) seeds. These proteins showed very similar molecular and kinetic properties but could be resolved by chromatofocusing and isoelectric focusing.

Plasma concentrations of clonazepam after single rectal administration.

Clonazepam was administered rectally to six children aged 1.4 to 4.7 years in a dose of 0.

Steroid responsive nephrotic syndrome is more common in Asians.

A retrospective study was undertaken to determine the incidence and compare the natural history of steroid responsive nephrotic syndrome in Asian, European, and Afro-Caribbean children born in the United Kingdom and living in Birmingham. Patients were identified from hospital admission records during the years 1979-83. Baseline population data were obtained from the same area using city birth statistics.