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PubMed1979

Characterization of Ca2+- and Sr2+-activated tension in functionally skinned chicken fibers of normal and dystrophic skeletal and normal cardiac muscle.

The Ca2+ and Sr2+ activation of tension in functionally skinned chicken fibers of normal and dystrophic skeletal and normal cardiac muscle were studied. The muscles studied can be separated into two groups based upon their Ca2+ and Sr2+ sensitivities: those which are significantly more sensitive to Ca2+ than to Sr2+, pectoralis and posterior latissimus dorsi (PLD), and those which show no Ca2+/Sr2+ sensitivity difference, cardiac and anterior latissimus dorsi (ALD). This suggests that there is more than one type of Ca2+ site involved in Ca2+ control of muscle contraction in different muscle types and suggests that ALD and cardiac muscle may be controlled by a different type of binding site than PLD and pectoralis muscle. Dystrophic ALD and PLD muscles showed little change in their Ca2+ and Sr2+ sensitivities from those of normal muscles in contrast to the pectoralis which showed a decrease in both Ca2+ and Sr2+ sensitivity (approaching that of PLD) with the onset of dystrophy. Similarly, upon SDS polyacrylamide gel electrophoresis, dystrophic ALD and PLD muscles showed no difference in contractile proteins from those of normal muscles, in contrast to pectoralis muscle where the appearance of a 36,000 dalton protein band correlated with the onset of dystrophy and the changes in the Ca2+/Sr2+ activation properties of this muscle. The contractile protein band pattern of normal and dystrophic PLD and dystrophic pectoralis muscle were similar including the presence of the 36,000 dalton protein.

Animals↗

PubMed1979

Independent development of contractile properties and myosin light chains in embryonic chick fast and slow muscle.

1. The contractile speeds and tetanus/twitch ratios of the slow anterior latissimus dorsi (ALD) and fast posterior latissimus dorsi (PLD) muscles were studied during embryonic development and correlated with the type of myosin light chains present in these muscles as studied by one and two dimensional polyacrylamide gel electrophoresis. 2. At a time when the contractions of PLD were slow, i.e. in 15 day old embryos, the myosin light chains in this muscle were of the fast type. The slow contraction of this muscle may be due to incomplete and slow activation of the contractile elements. The tetanus/twitch ratio of muscles from 15 day old embryos is low and increases sharply with age. This increase could be due to the maturation of the internal membrane system, and occurs at about the same time as the increase in the speed of contraction. 3. ALD muscles contract slowly during all stages of development, although their tetanus/twitch ratio also increases with age. At 13 days they contain a mixture of fast and slow type myosin light chains and with increasing age the proportion of the slow type myosin light chains increases at the expense of the fast type. The slow time course of contraction of ALD is consistent with the presence of slow type myosin light chains. 4. The possibility that the synthesis of the slow type myosin light chains in ALD is induced by early motor activity in chick embryos is discussed.

Age Factors↗

PubMed1977

Skin and conjunctival biopsies in adrenoleukodystrophy.

Conjunctival and skin biopsies were performed in an 11 1/2 year-old caucasian male affected by adrenoleukodystrophy (ALD). In Schwann cells surrounding myelinated axons in conjunctival and dermal nerve bundles, empty clefts and few arrays of lamellae were discovered. The vacuolization in the eccrine glands of the skin, another striking feature, has not been reported previously in ALD. The obtained results suggest that ALD can be diagnosed in skin specimens precluding major surgery for biopsy. They provide support to the hypothesis of Schaumburg et al. (1975) that ALD is a generalized metabolic disorder.

Adrenal Insufficiency↗

PubMed1979

Adrenoleukodystrophy and adrenomyeloneuropathy associated with partial adrenal insufficiency in three generations of a kindred.

Four cases of adrenoleukodystrophy (ALD) and one case of adrenomyeloneuropathy (AMN) have developed in a kindred over three generations demonstrating that AMN is a clinical variant of ALD. Pituitary-adrenal function studies were performed in 10 family members, including two affected males and four females identified as carriers of ALD/AMN. No pituitary-adrenal abnormality was found in the carriers. However, basal morning plasma adrenocorticotropic hormone (ACTH) levels were markedly elevated in the two males with ALD and AMN, despite the fact that they had no clinical signs of adrenal insufficiency and that morning plasma cortisol levels and their response to maximal exogenous ACTH stimulation appeared to be normal. In addition, the integrated 24-hour response to the administration were also subnormal in these two cases. Thus, people with ALD and AMN may have subclinical partial adrenocrotical insufficiency. No other endocrinologic dysfunction was identified.

Adolescent↗

PubMed1978

The effect of estriol succinate therapy on plasma renin activity and urinary aldosterone in postmenopausal women.

The effects of estriol succinate (Synapause, 2 mg daily) on the renin-aldosterone system and blood pressure (RR) were studied in 14 postmenopausal women after bilateral oophorectomy. Plasma renin activity (PRA) and daily urinary aldosterone excretion (dU-Ald) were determined 1 mth after the operation and before estrogen treatment, at the end of 2 mth therapy, and, for the third time, 2 mth after the termination of treatment with the drug. No changes in PRA, dU-Ald or RR were found in normotensive women, or in 3 women with hypertension in this group. Another group of 11 postmenopausal women was investigated after long-term estriol succinate therapy, which had lasted for 5-8 yr after oophorectomy. PRA, dU-Ald and RR were measured during treatment and 2 mth after terminating the therapy. No changes were found either in hormone differences in the mean levels of PRA or dU-Ald. The results suggest that estriol succinate is devoid of general harmful effects on the renin-aldosterone system during postmenopausal therapy for climacteric symptoms.

Aldosterone↗

PubMed1978

Mitochondrial enzyme activities in liver biopsies from patients with alcoholic liver disease.

The hypothesis that mictochondrial damage is a significant factor in the pathogenesis of alcoholic liver disease (ALD) was investigated by enzymic analysis of mitochondrial fractions isolated from needle biopsy specimens from control patients, patients with fatty liver due to chronic alcoholism, and from patients with other forms of liver disease. Enzymes associated with the inner and outer mitochondrial membranes showed normal levels in ALD. Enzymes associated with the mitochondrial matrix, glutamate dehydrogenase, malate dehydrogenase and aspartate aminotransferase showed significantly raised levels in ALD, but the levels in patients with non-alcoholic liver disease was normal. In addition, analysis of the mitochondria by sucrose density gradient centrifugation revealed no differences between control tissue and liver from patients with alcoholic liver disease. These results do not indicate that there is significant mitochondrial damage in ALD. The raised mitochondrial matrix enzymes may represent an adaptive response to the ethanol load.

Aspartate Aminotransferases↗

PubMed1979

Effects of ischemia on tissue metabolites in red (slow) and white (fast) skeletal muscle of the chicken.

Brief periods of ischemia have been shown to produce marked reactive hyperemia in both red (slow) and white (fast) skeletal muscle. However, evidence is lacking for specific vasodilator metabolites which are rapidly produced in ischemic skeletal muscle. The present study examined the effects of 1 and 3 minutes of ischemia on creatine phosphate (CrP), adenine nucleotide metabolism, and anaerobic glycolysis in red anterior (ALD) and white posterior latissimus dorsi (PLD) muscles of the chicken. Tissue metabolite concentrations were determined from perchloric acid or trichloroacetic acid extracts using enzymatic assay or high pressure liquid chromatography. CrP or adenine nucleotides were not significantly altered in either muscle following 1 or 3 minutes of ischemia. However, adenosine increased by 611% in the ALD at 1 minute. Following 3 minutes of ischemia, adenosine concentrations were elevated by 439% and 201% in the ALD and PLD, respectively. The PLD showed the greatest increases in inosine and IMP. Inorganic phosphate increased by 67% and lactate increased by 142% in the ALD at 3 minutes. The PLD, which is reported to have a high anaerobic glycolytic capacity, showed no increase in lactate. These results support the hypothesis that adenosine may be a mediator of akeletal muscle reactive hyperemia following short periods of ischemia.

Adenosine↗

PubMed2026

Dysregulation of the serum and IgG N-glycome in decompensated cirrhosis and its association with Model for End-Stage Liver Disease-Sodium (MELD-Na).

BACKGROUND AND AIMS: N-glycans modulate glycoprotein structure and function and are altered during chronic inflammation. We sought to define the extent of serum and IgG N-glycan disruption in patients with decompensated liver cirrhosis from alcohol-related liver disease (ALD), primary sclerosing cholangitis (PSC), and ALD-related hepatocellular carcinoma (HCC). Finally, we aimed to examine whether serum and IgG glycosylation is associated with changes in Model for End-stage Liver Disease-Sodium (MELD-Na) scores, a clinical marker used to prioritise liver transplantation. METHODS: Serum samples were obtained from patients with ALD (n = 17), PSC (n = 7), ALD-related HCC (n = 4), and healthy controls (n = 10). N-glycans were released, fluorescently labelled, and profiled by hydrophilic interaction ultra performance liquid chromatography (HILIC-UPLC). Chromatograms were integrated into 46 and 23 glycan peaks for serum and IgG respectively. These peaks and their associated glycosylation traits were statistically compared with healthy controls using age- and sex-adjusted linear regression models. RESULTS: In serum, decompensated cirrhosis shows statistically significant shifts toward less complex, agalactosylated and asialylated biantennary glycans, accompanied by significant losses of highly branched, galactosylated and sialylated structures. IgG mirrored this pattern, which is characteristic of a pro-inflammatory signature, with increased agalactosylation and bisected glycan levels, along with reduced levels of digalactosylated and sialylated species. N-glycan profiles showed significant associations with MELD-Na scores, indicating that inflammatory processes in decompensated liver cirrhosis continue to reshape serum glycoproteins. CONCLUSION: Decompensated liver cirrhosis shows profound remodelling of serum and IgG N-glycans. These data establish a reference framework for terminal glycomic disruption in liver disease and highlight the potential value of incorporating glycosylation analysis into broader assessments of liver disease progression.

Humans↗

PubMed1976

[Behavior of the plasma renin activity and aldosterone after salt restriction and upright posture in essential hypertension].

In 100 patients with essential hypertension and 50 healthy subjects the relation between plasma renin activity (PRA) and plasma aldosterone level (Ald) was investigated. PRA as well as plasma aldosterone was estimated a) after a diet with normal sodium content (equals 120 mEq per day) provided for 3 days and after an 8 hrs rest ("L" value of PRA and Ald) and b) after sodium restriction (10 mEq per day) for 3 days and 3-4 hrs ambulation ("A" value of PRA and Ald). Concerning the behaviour of "L"- und "A"-PRA all patients were divided into 4 groups: a) patients with normal, b) rigid, c) low and d) high PRA. Despite differentiated values of PRA in patients of all groups a normal increase of the plasma aldosterone level was stated after sodium restriction in the diet and upright position. Even in 18% of healthy subjects with low PRA a normal increase of Ald after sodium restriction and upright position was observed. These results seem to prove that in patients with low and rigid PRA and in 18% of healthy subjects the renin-angiotensin system is not the main or only regulator of aldosterone secretion.

Adult↗

PubMed1976

[Response of glutamic oxaloacetic transaminase, lactate dehydrogenase and aldolase in serum of cattle after transportation stress].

The response to transport stress of serum GOT, LDH, and ALD was studied in 35 heads of cattle, aged 18 months to 20 years. The influence of different factors upon serum enzyme behaviour in cattle after exposure to transport stress was tested by means of mathematico-statistical methods. The following conclusions were drawn: 1. Transport stress on cattle was followed by rise in GOT, LDH, and ALD activities in serum. 2. The strongest rise was recorded from ALD activity and the smallest from LDH. 3. All three serum enzymes continued to rise up to the time of slaughter (20 to 50 hours after transport). 4. The enzyme activities recorded from blood after slaughter did not differ significantly from those in venous blood. 5. Intensity of ALD activity rise was found to depend on the transport distance (if it was more than 20 km), age of animal, and ambient temperature. 6. The rise in GOT activity was found to depend only on the given ambient temperature. 7. The behaviour of LDH activity did not reveal any secured relationship with any of the examined factors (transport distance, age keeping, and tuberculosis state or ambient temperature).

Animals↗

PubMed2025

Alcohol-induced KDM5B activation in hepatocytes drives pathogenic cell-cell communication, leading to loss of liver function.

BACKGROUND: Alcohol-associated liver disease (ALD) is a major cause of alcohol-associated mortality. Previously, we identified KDM5B as a sex-specific mediator of ALD development; however, the mechanism behind KDM5B-induced pathological changes is not established. METHODS: Kdm5b flox/flox female mice were fed a western diet and 20% alcohol in the drinking water for 8-16 weeks (WDA). To induce KO, mice received 2×1011 genome copies of AAV8-CMV-Cre, AAV8-TBG-Cre, or AAV8-control. To test the role of myeloid C/EBPβ, Cebpbfl/fl, or Cebpbfl/fl Lyz2-Cre mice were fed WDA for 16 weeks. RESULTS: We found that Kdm5b KO prevented alcohol-induced liver fibrosis and liver inflammation in female mice. These changes were in part mediated by hepatocyte-to-non-parenchymal cell communication changes. KDM5B in hepatocytes promoted pro-inflammatory and pro-fibrotic changes in liver macrophages, endothelial cells, and stellate cells. Moreover, KDM5B promoted alcohol-induced early increase in EpCAM-positive liver progenitors and loss of liver function at later time points of alcohol feeding. We found that loss of liver function was dependent on a hepatocyte-to-macrophage communication feedback loop. KDM5B in hepatocytes inhibited macrophage C/EBPβ expression, which in turn resulted in loss of the mature KCs phenotype and prevented the ability of KCs to support hepatocyte differentiation, ultimately leading to loss of liver synthetic function. CONCLUSIONS: KDM5B activation in hepatocytes drives pathogenic cell-cell communication, leading to alcohol-induced loss of liver function in ALD.

Animals↗

PubMed1978

The role of muscle activity in the differentiation of neuromuscular junctions in slow and fast chick muscles.

The differentiation of neuromuscular junctions of multiply innervated, slow anterior latissimus dorsi (ALD) and focally innervated, fast, posterior latissimus dorsi (PLD) muscles was studied in normal and curarized chick embryos. At 16 days of incubation, fibres of both muscles are contacted by several axon profiles, the number of which falls with age. In 18-day-old embryos individual endplates in ALD are usually contacted by three axon profiles, whereas in PLD, endplates are contacted only by a single large terminal profile. At this time, there is already a significant accumulation of cell organelles in the postsynaptic area. Treatment of embryos with curare during the 7th and 12th day of incubation delays the differentiation of the neuromuscular junction in both muscles. The paralysis dramatically affects the decrease of the number of axon profiles at individual endplates in both muscles. At 16 days the number of axon profiles was greater in embryos treated with curare than in the untreated controls. At 18 days when the number of axon profiles normally decreases, the endplates of both types of curarized muscles have an even greater number of axon profiles than at 16 days. Endplates in curarized PLD had up to 13 and in curarized ALD up to 12 axon profiles. The effects of curare gradually wore off and when the movements of the embryos again became more vigorous, the normal differentiation of neuromuscular junctions continued. At 21 days of incubation many embryos recover from curare and show endplates of normal appearance in both muscles. These results suggest that activity of the muscle is essential for the maturation of the neuromuscular junctions.

Animals↗

PubMed1975

[Immunochemical studies on aldolase isoenzymes in human serum (author's transl)].

Analysis of human serum aldolase isoenzymes A, B and C activities was performed by means of a recently developed immunochemical assay. Isoenzyme patterns were established in 130 healthy subjects selected from a normal range research program. Total aldolase activity ranged from 0.8, 1.6, 2.5 U/L, Ald A from 0.6, 1.2, 1.9 U/L, Ald B 0.0, 0.2, 0.7 U/L, Ald C 0.0, 0.1, 0.4 U/L expressed as chi plus or minus 2 s range. Comparing the histograms of total aldolase activity in patients with acute hepatitis and normal controls, an almost identical frequency distribution in both groups was observed. However, histograms of aldolase isoenzyme B values of these groups were practically completely separated. Thus in contrast with total aldolase activity, the determination of aldolase isoenzyme B activity is a useful criterion in the diagnosis of acute hepatitis.

Antibodies↗

PubMed1977

Mercury induced cardiovascular abnormalities in the chicken.

Juvenile chickens were either chronically exposed to 300 microgram/ml of Hg in the drinking water (chronic dosage--CD) or acutely exposed to either 3 mg of Hg/kg body wt (acute low dosage--ALD) or 12 mg of Hg/kg body wt (acute high dosage--AHD) administered intramuscularly for five consecutive days. Only the CD and AHD treatments retarded normal growth. Relative heart weights were increased by the CD treatment, decreased by the AHD treatment, and remained unchanged during the ALD treatment. Relative aorta weights were increased by the CD treatment but decreased by the AHD treatment. ECG analyses revealed a consistent decrease in the amplitude of the R-S and T waves with the greatest effects present in the acute (ALD and AHD) treatments. Histological examination revealed that Hg caused myocardial histopathological changes characterized as a myocarditis with polymorphonuclear and lymphocytic infiltration and fatty degeneration. It is concluded tha Hg causes cardiovascular disturbances in chickens even when administered at levels which do not inhibit normal growth.

Animals↗

PubMed1979

EEG, pattern-evoked potentials and nerve conduction velocity in a family with adrenoleucodystrophy.

In a family with 8 children one case of adrenoleucodystrophy (ALD), verified by autopsy, 2 cases with clinical signs of ALD and four other clinically healthy subjects with pathological ACTH tests were found. As an initial sign in the EEG, temporo-occipital slowing occurred. Further EEG studies showed spread of the primary local abnormalities, indicating diffuse brain dysfunction during progression of the disease. Terminal EEG flattening may be characteristic for late stages of the disease. No correlation could be established between lateralization, paroxysms and actual stage of the disease. Abnormal EEGs occurred not only in clinically and subclinically affected male patients, but also in a girl, who might be a carrier of ALD. Visual evoked potentials showed an increased latency of the P2 wave in the clinically, and in one subclinically, affected subjects. Motor nerve conduction velocity was reduced in only 2 patients. Antidromic sensory nerve conduction velocity of the median nerve was normal in all patients.

Addison Disease↗

PubMed1979

Distribution of myosin isoenzymes among skeletal muscle fiber types.

Using an immunocytochemical approach, we have demonstrated a preferential distribution of myosin isoenzymes with respect to the pattern of fiber types in skeletal muscles of the rat. In an earlier study, we had shown that fluorescein-labeled antibody against "white" myosin from the chicken pectoralis stained all the white, intermediate and about half the red fibers of the rat diaphragm, a fast-twitch muscle (Gauthier and Lowey, 1977). We have now extended this study to include antibodies prepared against the "head" (S1) and "rod" portions of myosin, as well as the alkali- and 5,5'dithiobis (2-nitrobenzoic acid) (DTNB)-light chains. Antibodies capable of distinguishing between alkali 1 and alkali 2 type myosin were also used to localize these isoenzymes in the same fast muscle. We observed, by both direct and indirect immunofluorescence, that the same fibers which had reacted previously with antibodies against white myosin reacted with antibodies to the proteolytic subfragments and to the low molecular-weight subunits of myosin. These results confirm our earlier conclusion that the myosins of the reactive fibers in rat skeletal muscle are sufficiently similar to share antigenic determinants. The homology, furthermore, is not confined to a limited region of the myosin molecule, but includes the head and rod portions and all classes of light chains. Despite the similarities, some differences exist in the protein compositions of these fibers: antibodies to S1 did not stain the reactive (fast) red fiber as strongly as they did the white and intermediate fibers. Non-uniform staining was also observed with antibodies specific for A2 myosin; the fast red fiber again showed weaker fluorescence than did the other reactive fibers. These results could indicate a variable distribution of myosin isoenzymes according to their alkali-light chain composition among fiber types. Alternatively, there may exist yet another myosin isoenzyme which is localized in the fast red fiber. Those red fibers which did not react with any of the antibodies to pectoralis myosin, did react strongly with an antibody against myosin isolated from the anterior latissimus dorsi (ALD), a slow red muscle of the chicken. The myosin in these fibers (slow red fibers) is, therefore, distinct from the other myosin isoenzymes. In the rat soleus, a slow-twitch muscle, the majority of the fibers reacted only with antibody against ALD myosin. A minority, however, reacted with antiboddies to pectoralis as well as ALD myosin, which indicates that both fast and slow myosin can coexist within the same fiber of a normal adult muscle. These immunocytochemical studies have emphasized that a wide range of isoenzymes may contribute to the characteristic physiological properties of individual fiber types in a mixed muscle.

Adenosine Triphosphatases↗

PubMed1977

Differentiation of electrical and contractile properties of slow and fast muscle fibres.

1. The development of the electrical and contractile properties of slow anterior latissimus dorsi (ALD) and fast posterior latissimus dorsi (PLD) muscles of the chick was studied. 2. At the earliest ages studied (14-16 days of incubation), the membrane resistance was similar in both muscles. Subsequently the membrane resistance of PLD decreased, and that of ALD increased. Electrical differentiation continued after hatching. 3. The contractile speeds of ALD and PLD muscles were similar in embryos at 14-16 days of incubation, and suddenly differentiated at 17-18 days. Unlike the electrical properties, contractile speeds changed little after hatching. 4. It is suggested that the different electrical properties of the membrane of the two types of muscle fibre develop as a result of the particular type of excitation imposed by their nerves.

Action Potentials↗

PubMed1977

Reactive hyperemia in capillaries of red and white skeletal muscle.

Reactive hyperemia was examined in individual capillaries of the red anterior (ALD) and white posterior (PLD) latissimus dorsi muscles of the anesthetized chicken following total blood flow occlusion of 3.5-180 s in length. Capillary red cell velocities were measured by the dual-slit photometric technique. The control capillary velocities in the ALD and PLD were 0.56 +/- .03 (SE) and 0.34 +/- .03 mm/s, respectively. With increasing occlusion lengths, the reactive hyperemia peak velocity, hyperemia duration, and excess flow were found to increase progressively in both muscles suggesting a metabolic origin for the response. The ALD showed a greater peak reactive hyperemia flow than the PLD following occlusion lengths of 7-60 s and a greater excess flow following occlusion lengths of 15 s and longer. However, when these data were normalized to control velocities by calculating the peak-to-control velocity ratio and percent flow debt repayment, no significant differences were found between the two muscles. Furthermore, the reactive hyperemia durations were similar in the two muscles at each occlusion length. These data demonstrate that reactive hyperemia is similar in two muscle types which are known to differ considerably in resting metabolism and anaerobic capacity.

Animals↗