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Biomedical subjects

G Hübner

Publications and source records attributed to G Hübner.

At least 163 records · Page 9Linked to original sources
PubMed1981

[Body composition of light and heavy rats in a high or low fat diet group].

The authors studied the body composition of light and heavy male Wistar rats (approximately 11 and 21 weeks of age) from two diet groups fed a high-fat (HFD, 50 per cent (w/w) fat) and a low-fat diet (LFD, 3 per cent (w/w) fat), respectively. In both age-groups the heavy HFD rats showed a considerably higher fat proportion (as compared with light LFD rats) associated with a correspondingly reduced body water content and an approximately identical body protein concentration. On comparing light and heavy rats (11 and 21 weeks of age) from the same diet group as to their body composition, the authors found greater differences only in the 21-week-old HFD animals; as against light animals, the heavy animals exhibited a highly significant increase in body fat content and a highly significant decrease in body water content. A comparison of LFD and HFD animals of approximately the same weight revealed an increase of the relative body fat content and a decrease of the percentage of body water content in light 11-week-old and also in 21-week-old HFD animals as against heavy LFD animals.

Age Factors↗
PubMed1981

[Reducing body myopathy--ultrastructure and classification (author's transl)].

Reducing body myopathy is a very rare progressive muscular disease. Apart from a non-specific circumscribed muscle fiber degeneration it displays muscle fiber inclusions which have reducing properties as a typical morphological feature. The nosological classification of the disease is not settled. A case in a 15 years old girl is reported. She suffered from a progressive muscular weakness and atrophy and in a muscle biopsy regressive changes were obvious. Furthermore, multiple eosinophilic muscle fiber inclusions were also observed, which exhibited reducing properties. They consist of electron dense granules with a diameter of 12--16 nm. Since a sister of the patient has suffered for many years from a clinically and morphologically similar muscle disease, reducing body myopathy apparently belongs to the group of congenital myopathies with structural abnormalities. Muscle fiber inclusions with reducing properties also occur in another myopathy, in which they structural and histochemical differ widely from those found in reducing body myopathy. The reducing body myopathy is better named after its typical morphological property, the granular inclusions. It is proposed to name it "granular body myopathy".

Adolescent↗
PubMed1981

[Morphology and diagnosis of Zellweger syndrome. A contribution to combined cytochemical-finestructural identification of peroxisomes in autopsy material and frozen liver tissue with case report].

A female newborn, the second child of healthy non consanguineous parents, exhibited muscular hypotonia, areflexia, apathy, seizures, hepatomegaly and failure to thrive since birth. The peculiar skull shape was lacking. In the urine pipecolic acid and trihydroxycoprostanoic acid were excreted. At the age of seven weeks she died of bronchopneumonia. Lightmicroscopy revealed malformations and deficiency of myelinisation in the brain, renal cysts and fatty metamorphosis in the enlarged liver, which showed only minimal siderosis. Ultrastructurally no peroxisomes could be found in liver and kidney. No peroxisomes were detected by histochemical demonstration of catalase in frozen liver tissue which was taken immediately after death and stored for three months. Absence of peroxisomes is pathognomonic for the cerebro-hepato-renal syndrome of Zellweger and occurs in the liver irrespective of duration and degree of liver damage. It is best demonstrated by enzymehistochemical electron microscopy. With this method peroxisomes can be visualized even 30 h post mortem. In deep frozen normal liver tissue the activity of catalase remains very stable and enables the identification of peroxisomes even after a 12 months period of storage. In the cerebro-hepato-renal syndrome of Zellweger, frozen liver tissue should be stored for biochemical and diagnostic enzymehistochemical studies.

Autopsy↗
PubMed1981

[Enzymatic oxidation reaction in frozen parsley].

During frozen storage of (non-blanched) parsley, substances are formed showing absorption (234 nm) typical for hydroperoxidienoic acids with conjugated double bonds. However, only small quantities of these compounds are found in the frozen material as compared to other lipid degradation products; for instance, during 2 months at -18 degrees C only 0.1% of the fatty acids (bound to polar lipids) present in fresh parsley are transformed into dienoic acids. These low dienoic acid concentration are nevertheless sufficient to explain off-flavour formation since the sensory threshold values of the degradation products are in the range of some ppb only. After storage for 2 months at -24 degrees C no hydroperoxidienoic acids were found which means that this temperature, in contrast to the usual storage temperature of -18 degrees C, provides practically full protection against lipoxygenase-catalyzed spoiling reactions.

Food Preservation↗
PubMed1981

[Effectors and products of enzymatic diiodotyrosine deiodination by a plasmatic fraction from pig liver].

Effectors and products of enzymatic diiodotyrosine (DIT) deiodination by a cytosolic fraction of pig liver hab been investigated. 13% of the degraded 131I-DIT was found as monoiodotyrosine by thin layer chromatography. The main quantity of the deiodinated DIT was found on the start point of the chromatogram bound to enzyme protein. Tyrosine as a reaction product of enzymatic deiodination of [14C]-IT could not be identified exactly. The liver cytosolic deiodinase is activated by pyruvate; the extent of activation depends on th pyruvate concentration. Diiodohydroxyphenylpyruvate as a product of transamination and theoretically possible intermediate product could be excluded. NADPH 2 and sodium dithionite activated the deiodinase to 1/3, sodium dithionite together with FAD to 1/2 the amount of which was determined for the action of pyruvate. The enzymatic activity in the presence of pyruvate and NADPH2, respectively NADPH2/FAD is identical with the sum of the single activities. The effect of dithionite and sulfite on deiodinase activity depends on the concentration: low effector concentrations increase, while high concentrations decrease the enzyme activity. The liver plasma deiodinase was inactivate quantitatively by reaction with 10(-4) M PCMB; by reaction with 10(-4) M DTNB or NEM the inactivation was 40% only. The inactivation of deiodinase by PCMB was quantitative reversible by cysteine, while inactivation by DTNB was reversible by cysteine to maximal 70% only. Differences between cytosolic and microsomal deiodinases are discussed also in regard to the mechanism of DIT-deiodination by a liver cytosolic fraction with direct participation of SH-groups.

Animals↗
PubMed1980

[Myocardial involvement in Fabry's disease (author's transl)].

Definitely impaired coronary reserve, as recorded in cases with coronary microangiopathy, was demonstrated in a 48-year-old female non-smoker with angina, positive ECG exercise tolerance test and negative coronary angiography with normal coronary circulation at rest. Myocardial biopsy from the right ventricle demonstrated electron-microscopically the lamellar, intracytoplasmic inclusion bodies described in Fabry's disease. This diagnosis of an X-chromosomal recessive metabolic disorder with prominent myocardial involvement was confirmed by the results of further investigations.

Angina Pectoris↗
PubMed1980

[Cholesterol esterification in the serum of hypothyroid rats].

The ratio of unesterified to total serum cholesterol in hypothyreotic rats has been studied. Extreme rat hypothyreosis of identical degree was induced by radiothyroidectomy (group R-HT) and feeding of thyreostatics (groups MTU-HT and MMI-HT). Determination of total serum cholesterol content and the unesterified cholesterol content resulted in following changes (all changes significant for p < 0,001): 1. Higher total cholesterol in hypothyreotic rats 4 and 7 weeks after beginning the experiments as compared to the controls. 2. The ratio of unesterified cholesterol to total cholesterol was increased in all hypothyreotic groups. It is discussed, that the esterification reaction in serum catalyzed by the lecithin-cholesterol-acyltransferase (LCAT) is diminished in the hypothyreotic state.

Animals↗
PubMed1980

[The lactate-pyruvate quotient in the framework of modern thyroid diagnosis].

In literature a big number of central and peripheral parameters for the diagnosis and control of therapeutical measures in diseases of human thyroid gland is described. That means, that none of the methods used has a sufficient weight. The enzymatic estimation of pyruvate and lactate reflect the peripheral effect of the hormones of thyroid gland. The pyruvate and lactate levels differ in the several groups of patients with euthyreosis, hypothyreosis, hyperthyreosis and euthyreotic goitre. Between these all groups the lactate/pyruvate ratio showed highly significant differences. The comparison of the lactate/pyruvate ratio and the T3-values showed highly significant correlation between both parameters. This good conformity is underlined by the behaviour of the parameters in courses of therapy, with additional support by farther functional tests--ETR-, T3- and T4-test. Our results confirmed the suggestion, also in agreement with literature, that the lactate/pyruvate ratio represent a possible enrichment in the laboratory programme for diagnosis and control of therapy in patients with thyroid diseases.

Adolescent↗
PubMed1980

Wolman's disease: clinical, biochemical and ultrastructural studies in an unusual case without striking adrenal calcification.

A case of Wolman's disease is described in a German infant who died at the age of 4 months. Hepatosplenomegaly, abdominal distention, gastrointestinal symptoms, dyserythropoietic changes in the bone marrow, but not adrenal calcification on X-ray were present. Stored lipid material could be demonstrated in liver, spleen, intestine, adrenals, thymus, kidneys, blood cells, but not in the central nervous system. Cholesterylesters and triglycerides were markedly increased in liver and spleen. Lysosomal acid lipase was found to be decreased in leucocytes and liver to less than 10% of normal, when measured with synthetic and natural substrates.

Adrenal Glands↗
PubMed1980

[Ultrastructural and clinical studies in "inclusion body myositis" (author's transl)].

A non-hereditary slowly progressive neuromuscular disease occuring in a 39-year-old male, consisting of an asymmetrical wasting of the muscles of the shanks, is reported. Neither clinical nor electromyographic criteria can safely distinguish whether a primary neurogenic or a primary myogenic process is causally involved. The initial rise in serum CPK-activity indicates a myopathic origin. The lightmicroscopic aspect of the muscle-biopsy shows a muscular atrophy which resembles a primary neurogenic pattern with grouped clusters of atrophic fibers as well as a myopathic or rather myositic tissue-pattern with randomly scattered atrophic fibers. In addition there are numerous vacuoles which are equivalent to concentric lamellae of dense material in electronmicroscopy. Numerous fibrillary inclusions as well in the cytoplasm as in the cell-nucleus correspond to typical alterations in the so-called "inclusion body myositis". The disease is critically discussed as a nosological entity among neuromuscular disorders.

Adult↗
PubMed1980

[Liver deiodinase activity in rats after high and low fat iodine deficient diets].

Male Wistar rats were restricted on iodine intake by feeding pure diets, without iodine addition. The high fat diet contained 50% fat (HFD), the low fat diet 3% fat (LFD). Control animals received the same diets, but with iodine addition. After feeding the diets for 7 or 19 weeks liver deiodinase activity was estimated. The iodine deficient animals showed a diminished enzyme activity in both diet groups. But after feeding the diet without iodine addition for 7 weeks the deiodinase activity significantly decreased in the HFD-group only in relation to liver weight per 100 g body weight. After 19 weeks the liver deiodinase activity in the HFD-group was diminished significantly, but the decrease was much smaller than in the LFD-group. The estimation of relative thyroid weight and iodoamino acid distribution demonstrate that the level of iodine deficiency was in iodine restricted HFD-rats greater than in appropriate LFD-rats. This result was verified by investigations of thyroidal radio iodine uptake and iodine content, PB131I, serum iodine and T4 levels in both diet groups. The reason for the lower decrease of deiodinase activity at higher iodine deficiency in HFD-rats as compared to LFD-rats and the possible importance of food enriched with fat in endemic goiter areas is discussed.

Animals↗