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P J Pretorius

Publications and source records attributed to P J Pretorius.

At least 37 records · Page 2Linked to original sources

The correlation between biochemical and histopathological findings in adrenoleukodystrophy.

Nine areas of the brain from a case of adrenoleukodystrophy were examined histopathologically and by gas chromatography for fatty acid content. The main findings were: (1) the degree of demyelination was related to the pentacosanoic and hexacosanoic to docosanoic acid ratios (C25:0/C22:0 and C26:0/C22:0); gliosis was related to the ratios of several fatty acids to docosanoic acid; (2) there was a shift towards smaller components (C22:0, C23:0, C24:0) of the saturated fatty acid series in the less affected areas, to larger components and various minor components in regions of active demyelination; (3) mainly saturated fatty acids of the middle class components (C24:0, C25:0 and C26:0) were found in severely affected areas where the active process is complete. Because a region of high long chain fatty acid content, lacking histopathological change, was detected, the hypothesis is presented that the primary event in childhood ALD is related to defective lipid metabolism and that this preceeds demyelination.

Adrenoleukodystrophy↗

Biochemical evidence for clinically diagnosed adrenoleucodystrophy in two brothers.

Adrenoleucodystrophy (ALD) is a rare, X-linked inherited disease affecting the central nervous system. The clinical findings overlap those of other related progressive neurological diseases and complicate the diagnosis. In this article biochemical analyses of the very-long-chain fatty acid distribution in specimens obtained from 2 related patients clinically diagnosed as having ALD, which unequivocally confirmed the diagnosis, are described. Analyses of the long-chain fatty acids were performed on postmortem brain tissue samples from the one patient. In the other, who is in the terminal phase of the disease, the long-chain fatty acids were analysed in plasma, erythrocytes and fibroblasts from tissue culture. The limitations of the different analyses for biochemical identification of ALD are discussed.

Adrenoleukodystrophy↗

Cardiovascular origins of heatstroke pathophysiology: an anesthetized rat model.

Opinion is divided on issues concerning a cardiovascular origin of heatstroke pathophysiology as well as those concerning the mechanism of circulatory failure in heat. Such controversies seem to arise because of insufficient data covering the prodromal period of heatstroke. A reappraisal of the nature and sequence of early circulatory responses to heat stress was, consequently, undertaken by subjecting male albino rats to an environmental temperature of 45 degrees C at 15% relative humidity until overt circulatory failure occurred. Although the initial circulatory responses were normal for mild exertion, an inapparent circulatory crisis developed, probably a result of the abolishment of compensatory splanchnic vasoconstriction. The impending threat of functional hypovolemia was, temporarily at least, obscured by cardiac compensation and the extent of cardiovascular commitments may, therefore, have been underestimated. This also suggests that cardiovascular adjustments during the stress of heat may prove to be an essential feature of heatstroke pathophysiology.

Animals↗

A study on the function of some subcellular systems of the sheep myocardium during gousiekte. I. The energy production system.

In order to determine the status of the energy production system of the heart during cardiac failure of sheep with gousiekte, observations were made of the heart tissue levels of adenosine triphosphate (ATP), creatine phosphate (CrP), inorganic phosphate, reduced nicotine adenine dinucleotide (NADH) and lactate. Some measurements on oxidative phosphorylation were also made. A significant decrease in ATP and CrP levels coincided with a simultaneous rise in the ATP:CrP ratio and lactate levels in gousiekte hearts. No significant deviations in inorganic phosphate and NADH levels could be demonstrated. These abnormalities were accompanied by a decreased uptake of oxygen by isolated mitochondria of gousiekte hearts. There was a marked increase in the anaerobic state of the hearts of dying gousiekte sheep, while the values of NADH and the ATP:CrP ratio at a presymptomatic stage indicated a possible early derangement in the energy metabolism of sheep fed the toxic material. No hypertrophy could be detected for the failing ventricles of gousiekte sheep after being corrected for a significant amount of oedema found in the heart tissue of these animals. It was concluded that the depressed ATP and CrP levels in the heart tissue of gousiekte sheep during cardiac failure could at least in part, be attributed to a depressed aerobic energy production. It is not possible, however, to state whether this is a primary or a secondary response due to intoxication and also whether it could be seen as a cause or effect of cardiac failure.

Adenosine Triphosphate↗

A study on the function of some subcellular systems of the sheep myocardium during gousiekte. II. The contractile protein system.

Two groups of Merino sheep were intoxicated separately and at different times with "gousiektebossie" (Pachystigma pygmaeum) until definite symptoms of heart failure were auscultated. Cardiectomy was carried out and some ventricular muscle from 1 group was stored in 50% glycerol at -20 degrees C for about 4 months. Natural actomyosin (n-actomyosin) was subsequently extracted and tested for magnesium, calcium and adenosine triphosphate (ATP)-dependent adenosine triphosphatase (ATP-ase) activity as well as for superprecipitation characteristics. Muscle strips were taken from the other group and stored for 2 weeks in 50% glycerol at -20 degrees C, whereafter it was analysed for an isometric tension-calcium response. The data showed no difference between gousiekte and control sheep in the sensitivity of the contractile system to the activating effect of calcium ions with respect to isometric tension development. A significant reduction of the magnesium dependent ATP-ase was found for gousiekte n-actomyosin in either the presence or absence of calcium ions. A depressed sensitivity for this enzyme to increasing concentrations of ATP in comparison to controls was also found ([ATP] less than 1 mM, [MgCl2] = 1 mM). No significant difference could be detected in the sensitivity of the n-actomyosin:ATP-ase system to magnesium. n-Actomyosin:ATP-ase of gousiekte hearts revealed a depressed sensitivity to calcium ions. Gousiekte n-actomyosin also showed a significant depression in the rate of superprecipitation with a concomitant increase in the duration of the clearing phase. We conclude from these observations that a definite biochemical lesion is induced in the contractile proteins of heart muscle obtained from sheep intoxicated with "gousiektebossie" at the stage of cardiac failure. This condition is characterized by abnormal superprecipitation characteristics and a depressed n-actomyosin:ATP-ase activity, showing a reduced sensitivity to the activating effect of calcium ions.

Actomyosin↗

Changes in polyribosomes and poly(A)-containing polyribosomal RNA in the uterus of the ovariectomized rat in response to oestradiol-17 beta treatment.

Polyribosome formation and the characteristics of polyribosomal poly(A)-containing RNA from uteri of ovariectomized rats responding to a single dose of oestradiol-17 beta was investigated. The mean proportion of polyribosomes in the atrophic uterus was 65%. In response to 10 micrograms of oestradiol-17 beta/100 g body mass, the amount of polyribosomes increased to 88% 24 h after stimulation. Thereafter the proportion of polyribosomes decreased to a value of 48% at 72h. The pattern of amino acid incorporation in oocytes from Xenopus laevis injected with these polyribosomes was similar to the changes in polyribosome formation and degradation. The polyribosomal poly(A)-containing RNA from the controls consisted of a heterogeneous population of RNA with sedimentation values between 5S and 25S. The hormone stimulation resulted in an increase in both the amount and the size (13S to 35S) of the RNA.

Animals↗

[William Harvey (2 April 1578)--400 years of physiology].

William Harvey, born on 2 April 1578, was the founder of modern scientific physiology. The importance of his work was that he was the first prominent exponent of the tremendous value of experimental physiology. By postulating the machine model of the circulatory system he also reinforced the idea of Descartes that man is a machine. During the past 400 years this model led to sensational discoveries on the one hand, as well as scientific limitations and cultural and spiritual crises on the other. Serious rethinking about prescientific and prephilosophic premises is urgently needed for the future.

Blood Circulation↗