Publications by authors named "BLASS J"

Thiamine deficiency is a useful animal model of the interaction between biochemistry and behavior. Although numerous biochemical changes have been detected in thiamine deficiency, studies of behavioral changes are relatively scarce. We have modified and quantitated the string test, originally described by Miquel and Blasco, for application to thiamine-deficient rats.

View Article and Find Full Text PDF

1 - Protamine was incubated "in vitro", with human plasma and serum, and the initial degradation products (1-4 hours at 30 degrees C) were characterized by paper chromatographic and paper electrophoretic methods. The analysis revealed the presence of the arginine and also, in low quantities, that of a supplementary compound, an arginine peptide. Comparative experiences with the same substrate revealed that carboxypeptidase B liberated exclusively the C-terminal arginine, plasmin exclusively peptides of arginine, and trypsin, besides arginine peptides, minutes quantities of free arginine.

View Article and Find Full Text PDF
Food selection in the aged.

Int J Obes

December 1980

Old people's eating habits are often idiosyncratic and unhealthy; their feeding can reflect such brain disease are dementia, depression, paranoia; poor nutrition can contribute to degenerative brain disease. The modification of nutritional requirements of the aged by genetic factors is discussed.

View Article and Find Full Text PDF

Cross reactions investigated by double immunodiffusion and by immunoelectrophoresis between human plasma and plasma of 5 animal species using a specific rabbit antiserum against human At-III revealed antigenic relationships between the antithrombins of these species. But the quantitative measurements of At-III by immunologic methods (electro-immunodiffusion and radial immunodiffusion), using the rabbit antiserum, were not possible in common laboratory animals, except in baboons.

View Article and Find Full Text PDF

The authors study the role of thiamine-deficient diets in genetic variations contributing to Wernicke-Korsakoff syndrome.

View Article and Find Full Text PDF

Physostigmine improved videotape scores of ataxia in patients with various inherited ataxias. This improvement occurred in 12 out of 12 patients when the drug was given as a single dose and in nine of 11 patients when the drug and placebo were given in a long-term, double-blind randomized trial. Patients report various mild to moderate clinical benefits after the sustained use of physostigmine for 6 to 36 months.

View Article and Find Full Text PDF

There is now a great deal of evidence to link genetic defects of pyruvate metabolism to brain disease. Experimental evidence is reviewed in Chapter 12, and clinical evidence has been reviewed above. Severe lesions of components of the pyruvate dehydrogenase complex are associated with severe generalized brain disease, and milder defects with inherited ataxias.

View Article and Find Full Text PDF

We studied a thiamine-requiring enzyme in cultured cells from four patients with the Wernicke-Korsakoff syndrome to determine whether these patients have a genetic predilection to thiamine deficiency. Transketolase in fibroblasts from the patients with the syndrome bound thiamine pyrophosphate less avidly than control lines. The apparent Km for thiamine pyrophosphate was 195 +/- 31 micron for transketolase in extracts of the patients' cells as compared to 16 +/- 2 micron in six control lines (means +/- S.

View Article and Find Full Text PDF

Heparin cofactor activity as measured by an amidolytic method has been investigated in 48 patients undergoing cardiac surgery with extra-corporeal circulation (E.C.C.

View Article and Find Full Text PDF

Biochemical studies of psychiatric patients have revealed many abnormalities but have failed to identify specific biochemical changes linked to the pathophysiology of the illnesses. Review of these data in the light of newer concepts of psychiatric diagnosis may help to clarify the relationship between biochemical abnormalities and disabilities. These concepts may also suggest useful directions for future research.

View Article and Find Full Text PDF

Radiochemical methods are described in detail to measure the activities of the pyruvate dehydrogenase complex and of the ketoglutarate dehydrogenase complex in platelet-enriched fractions. Determinations can be completed in one day with as little as 5 ml of venous blood. Activities are proportional to the length of the incubation and the amount of tissue protein added, show appropriate dependence on added cofactors, are stable for up to 2 days at -20 degrees C, and do not appear to be affected by diet.

View Article and Find Full Text PDF

Physostigmine was given to 12 patients with various spincerebellar degenerations, and neurologic examinations were recorded on video tapes and assessed on a semiquantitative scale. Forty minutes after a single dose, the scores improved by 35.7 +/- 4.

View Article and Find Full Text PDF

Two brothers, aged 11 years 6 months and 2 years 3 months, with psychomotor and growth retardation, episodes of weakness, ataxia, ophthalmoplegia, and elevated levels of blood pyruvate were shown to have a deficiency in the pyruvate dehydrogenase complex (PDH). When they ate a diet high enough in fats to cause ketonemia but not acidosis, there was a fall in blood pyruvate levels, a decrease in the frequency and severity of the episodes of neurological deterioration, an increased rate of growth and development in the younger brother, and increased strength and endurance in the older one. The possibility of dietary treatment makes the early diagnosis of PDH deficiency more important.

View Article and Find Full Text PDF

A Canadian Indian family is described in which three of the children were mentally retarded, and had seizures and other neurological abnormalities. They had chronic metabolic acidosis associated with elevated blood levels of lactate, pyruvate, and alanine. Two of the children excreted large amounts of pyruvic and alpha-ketoglutaric acids in the urine and had elevated plasma levels of glutamic acid and proline.

View Article and Find Full Text PDF

A 9-year old boy with severe mental and growth retardation and diffuse neurologic damage had minimal elevation of blood pyruvate (0.21 mM) and lactate (2.1 mM) on a normal diet but developed life-threatening lactic acidosis (pH 7.

View Article and Find Full Text PDF