scholarly journals Hepatic storage of oligosaccharides and glycolipids in a cat affected with GM1 gangliosidosis

1978 ◽  
Vol 175 (3) ◽  
pp. 945-953 ◽  
Author(s):  
E W Holmes ◽  
J S O'Brien

1. Glycopeptides and glycolipids were isolated from normal cat liver and liver from a cat affected with GM1 gangliosidosis. 2. Bio-Gel P-6 chromatography of the crude glycopeptide fractions demonstrated three major peaks of hexose-containing compounds that were greatly increased in the mutant liver sample; these peaks contained oligosaccharides that comprised over 2% of the liver wet weight. 3. Two of the major pathological oligosaccharides, GP5 and GP6, were purified by chromatography on charcoal/Celite and Sephadex G-25. Oligosaccharides GP5 and GP6 had apparent mol.wts. of 1800 +/- 200 and 1350+/-200 respectively, and contained galactose, mannose and N-acetylglucosamine in molar proportions of 2.0:3.1:4.1 (GP5) and 1.0:2.2:2.7 (GP6). Periodate oxidation studies demonstrated the presence of galactose in a non-reducing terminal position. 4. The neutral glycolipid fraction from the mutant cat liver has a 1.3-fold increase in hexose content accompanied by an increased concentration of asialo-(ganglioside GM1). 5. There was a 2-fold increase of gangliosides in the mutant cat liver compared with normal cats. Ganglioside GM1 and a compound tentatively identified as N-glycolloyl-(ganglioside GM1) were the major glycolipids accumulated.

Development ◽  
1981 ◽  
Vol 62 (1) ◽  
pp. 325-338
Author(s):  
Elizabeth J. Thornber ◽  
Marilyn B. Renfree ◽  
Gregory I. Wallace

The in vitro uptake and incorporation of [3H]ui idine by blastocysts of the tammar wallaby showed a 16- and 30-fold increase from day 0 to day 10 after removal of pouch young, respectively. Two of the six non-expanded blastocysts recovered on day 5 showed a tenfold increase in incorporation. During the first ten days after removal of pouch young the diameter of the blastocyst increased threefold. Endometrial exudate from gravid uteri had a higher protein concentration than exudate from nongravid uteri (39·5 ± 0·9 and 32·0 ± 2·0 mg/ml (mean ± s.e.m.), respectively). Endometrial exudates from uteri where the blastocyst was actively growing were found to contain six uterine-specific proteins. These were separated by gradient polyacrylamide gel electrophoresis. Two of the proteins were pre-albumins and the others were larger molecules (M.W. 153000–670000). Two proteins were only present at particular stages of pregnancy: the other four were present at all stages from diapause to birth, in exudate from gravid and nongravid uteri. The specific binding of progesterone and androstenedione to proteins in endometrial exudates or uterine flushings from pregnant wallabies was less than one per cent of the value obtained from day-5 pregnant rabbits. The ability of mouse blastocysts to take up and incorporate [3H]uridine into acidinsoluble material increased threefold in the presence of day-10 endometrial exudates from wallabies. However, this was less than ten percent of the values obtained in the presence of bovine serum albumin. The concentration of calcium in endometrial exudates increased from 23·6 to 45·2 μg/ml during pregnancy; in endometrium it remained at 88·7 μg/g (wet weight) throughout pregnancy, and in plasma it was 53·3 μg/ml. The concentration of zinc in endometrial exudates was 4·5 μg/ml; in endometrium it decreased from 21·8 to 13·3 μg/g (wet weight) during pregnancy and in plasma it was 0·6 μg/ml.


1984 ◽  
Vol 62 (12) ◽  
pp. 1341-1347 ◽  
Author(s):  
Duncan Stewart ◽  
Elaine Pountney ◽  
David Fitchett

Norepinephrine-stimulated prostacyclin synthesis was studied in rat aortic rings by measuring 6-keto-prostaglandin F1α (6-keto-PGF1α) by radioimmunoassay. Norepinephrine (10−6 M) results in a 10- to 20-fold increase in 6-keto-PGF1α synthesis by rat aortic rings (54 ± 11 to 437 ± 260 pg∙mg wet weight−1∙20 min−1). The maximal stimulation of 6-keto-PGF1α synthesis was observed with a norepinephrine concentration of 10−5 M at a mean effective concentration (EC50) of 9.5 ± 3.2 × 10−7 M which is similar to the contractile response (Emax = 10−5 M, EC50 = 6.5 ± 1.8 × 10−7 M). Potassium chloride (30 mM), although causing a similar maximal contractile response as 10−6 M norepinephrine, did not increase 6-keto-PGF1α synthesis. Norepinephrine-stimulated 6-keto-PGF1α synthesis was dependent upon extracellular calcium. Norepinephrine stimulation in Ca2+-free medium did not lead to a significant increase in 6-keto-PGF1α synthesis. However, on the introduction of Ca2+, 6-keto-PGF1α synthesis was restored to its initial level. Phentolamine (10−6 M) (an α-adrenergic antagonist) and trifluroperazine (2.5 × 10−4 M) (a calmodulin inhibitor) completely inhibited norepinephrine-stimulated 6-keto-PGF1α synthesis, whereas verapamil 3 × 10−6 M (a calcium channel blocking drug) only partially inhibited synthesis (control, 74 ± 12; norepinephrine, 437 ± 260; norepinephrine + verapamil, 123 ± 8 pg∙mg wet weight−1∙20 min−1). Norepinephrine-stimulated prostacyclin synthesis is probably α-receptor mediated and appears to depend upon the opening of receptor-operated Ca2+ channels. The operation of potential-dependent Ca2+ channels (by 30 mM KCl) in contrast, although known to be associated with an increase of intracellular Ca2+, does not stimulate PG synthesis.


1978 ◽  
Vol 43 (2) ◽  
pp. 127-131 ◽  
Author(s):  
Yoshiyuki Suzuki ◽  
Norimasa Nakamura ◽  
Kazuko Fukuoka

2010 ◽  
Vol 22 (9) ◽  
pp. 119
Author(s):  
Y. Soh ◽  
L. J. Parry

Aquaporins (AQPs) have been implicated in the regulation of fluid balance in the cervix during pregnancy to promote hydration, a characteristic of cervical ripening in late gestation.There are four AQPs in the cervix; AQP3, 4, 5 and 8. Cervical fluid balance involves AQP5 and 8 in early pregnancy and AQP3 and 4 in late pregnancy [1]. However, the factors involved in the regulation of cervical AQPs are unknown. We propose that the ovarian peptide hormone relaxin regulates cervical AQPs because high circulating levels of relaxin correspond to changes in AQPs and it is involved in cervical ripening. To test this hypothesis, expression of aqp3, 5 and 8 was compared in the cervices of relaxin wildtype (Rln+/+) and relaxin knockout (Rln–/–) at various stages of pregnancy (day 14.5, 16.5 and 18.5 pregnancy) by quantitative PCR. In the Rln–/– mice, aqp3 expression was significantly lower on day 18.5 gestation compared to Rln+/+ littermates. Aqp5 and 8 expression did not change significantly between genotypes. To determine whether relaxin could restore the Rln+/+ phenotype, Rln–/– mice were implanted with Alzet osmotic minipumps on day 12.5 pregnancy to infuse either recombinant H2 human relaxin (200 μg/mL; Corthera Inc) or 0.9% saline as a control. Cervices were collected after 4 or 6 days of infusion for gene expression analysis. Relaxin infusion in pregnant Rln–/– mice increased cervical aqp3, and also decreased aqp5 expression compared with saline-controls in the 6-day infusion group. Additionally, relaxin treatment caused a 6-fold increase in cervix wet weight, dispersal of collagen fibres and a decrease in relaxin receptor (Rxfp1) expression. These data suggest that relaxin promotes cervical hydration through an action on AQPs. (1) Anderson et al, 2006. Endocrinology 147(1): 130–140.


1970 ◽  
Vol 120 (1) ◽  
pp. 95-103 ◽  
Author(s):  
Janet M. Wimhurst ◽  
K. L. Manchester

1. Measurements of the activities in rat liver of the four key enzymes involved in gluconeogenesis, i.e. pyruvate carboxylase (EC 6.4.1.1), phosphoenolpyruvate carboxykinase (EC 4.1.1.32), fructose 1,6-diphosphatase (EC 3.1.3.11) and glucose 6-phosphatase (EC 3.1.3.9), have been carried out, all four enzymes being measured in the same liver sample. Changes in activities resulting from starvation and diabetes have been studied. Changes in concentration (activity/unit wet weight of tissue) were compared with changes in the hepatic cellular content (activity/unit of DNA). 2. Each enzyme was found to increase in concentration during starvation for up to 3 days, but only glucose 6-phosphatase and phosphoenolpyruvate carboxykinase showed a significant rise in content. Fructose 1,6-diphosphatase appeared to decrease in content somewhat during the early stages of starvation. 3. There was a marked increase in the concentration of all four enzymes in non-starved rats made diabetic with alloxan or streptozotocin, for the most part similar responses being found for the two diabetogenic agents. On starvation, however, the enzyme contents in the diabetic animals tended to fall, often with streptozotocin-treated animals to values no greater than for the normal overnight-starved rat. Deprivation of food during the period after induction of diabetes with streptozotocin lessened the rise in enzyme activity. 4. The results are compared with other published values and factors such as substrate and activator concentrations likely to influence activity in vivo are considered. 5. Lack of correlation of change in fructose 1,6-diphosphatase with the other enzymes questions whether it should be included in any postulation of control of gluconeogenic enzymes by a single gene unit.


1973 ◽  
Vol 51 (11) ◽  
pp. 1544-1547 ◽  
Author(s):  
Bruce G. Berman ◽  
Mitchell L. Halperin

This study was designed to investigate the relation between activation of pyruvate dehydrogenase in white adipose tissue and the intracellular pyruvate content. The intracellular pyruvate level was approximately 8 nmol/g wet weight when adipose tissue was incubated up to 20 min with fructose (1 mg/ml) as substrate. The addition of insulin to the incubation medium resulted in a 1.5-fold increase in the active form of pyruvate dehydrogenase after 20 min incubation without raising the intracellular pyruvate level at earlier time periods. Intracellular pyruvate contents were increased 1.5-fold by TMPD (75 μM), but in this case there was no concomitant activation of pyruvate dehydrogenase. We conclude that the insulin-induced activation of pyruvate dehydrogenase is caused by mechanism(s) in addition to an elevation of pyruvate concentration.


Gene Therapy ◽  
2003 ◽  
Vol 10 (17) ◽  
pp. 1487-1493 ◽  
Author(s):  
N Takaura ◽  
T Yagi ◽  
M Maeda ◽  
E Nanba ◽  
A Oshima ◽  
...  

2008 ◽  
Vol 20 (9) ◽  
pp. 44
Author(s):  
Y. M. Soh ◽  
L. A. Vodstrcil ◽  
L. J. Parry

The cervical extracellular matrix (ECM) changes extensively in a process called cervical ripening in late pregnancy. This occurs through a complex series of processes leading to collagen fibre degradation and dispersal. We tested the hypothesis that relaxin increases cervical water content by stimulating hyaluronic synthase (HAS) enzymes that regulate hyaluronic acid (HA) synthesis. HA is thought to recruit water molecules into interstitial spaces between collagen fibrils, causing them to disperse. In the first study, cervices were collected from Rln+/+ and Rln−/− mice on days 14.5, 16.5, 18.5 and 19 gestation, and day 1 postpartum (PP) to compare has expression between the genotypes by qPCR analysis. In the second study, Rln−/− mice were implanted with Alzet osmotic minipumps on day 12.5 gestation to infuse either recombinant H2 human relaxin (200 μg/mL; BAS Medical Inc.) or 0.9% saline at a continuous flow rate of 0.5 μl/h. Cervices were collected after 4 or 6 days of infusion. has1 and has2 gene expression increased significantly at term and decreased immediately postpartum in the Rln+/+ mice. Although has1 and has2 were expressed in Rln−/− mice, there was no increase in expression on day 18.5 gestation and cervix wet weight did not increase compared with Rln+/+ mice. Relaxin infusion for 4 or 6 days in pregnant Rln−/− mice significantly increased cervical has2 but decreased has1 expression compared with saline-controls. Additionally, relaxin treatment caused a 6-fold increase in cervix wet weight, a 5% increase in water content and a significant decrease in relaxin receptor (Rxfp1) expression compared with saline-controls. These data suggest that relaxin promotes cervical hydration through an has2-mediated increase in HA, and may facilitate cervical ripening by causing collagen fibril dispersal in the ECM.


1985 ◽  
Vol 59 (1) ◽  
pp. 24-27 ◽  
Author(s):  
F. E. Kuhn ◽  
S. R. Max

The effects of chronic treatment with testosterone propionate on compensatory muscle hypertrophy secondary to synergist removal were studied in female rats. Synergist removal resulted in a significant (2-fold) increase in muscle wet weight, with no changes in protein concentration. As reported previously, oxidation of [2–14C]pyruvate to 14CO2 was significantly decreased in hypertrophic muscles. In addition, malate dehydrogenase and lactate dehydrogenase activities were significantly decreased in overloaded muscles on a wet weight basis but not on the basis of noncollagen protein. These data suggest that specific metabolic adaptations may occur in response to overload of muscle. Administration of testosterone propionate in subcutaneously implanted Silastic capsules resulted in a 20-fold increase in serum testosterone levels. This treatment had no effect on body weight, muscle weight, pyruvate oxidation, or malate and lactate dehydrogenase activities in both control and hypertrophic muscles, although there was an effect on the noncollagen protein content of overloaded muscles. These results do not support the hypothesis that androgens, in conjunction with weight-bearing exercise in female subjects, are effective in increasing muscle mass or function in female subjects.


2021 ◽  
Vol 70 (1-2) ◽  
pp. 11-14
Author(s):  
Miroslava Polovinski Horvatović ◽  
Ivan Radović ◽  
Igor Jajić ◽  
Saša Krstović ◽  
Mile Mirkov

Summary The purpose of this research is to investigate the occurrence of two heavy metals (namely cadmium (Cd) and lead (Pb)) in the liver of hogs bred in different locations in Vojvodina. A total of 30 liver samples were collected from ten pig farms in Vojvodina for experimental purposes in the period from December 2017 to January 2018. The samples collected were analysed for the presence of lead and cadmium. The average concentration of lead in all the samples was 0.39 mg/kg wet weight, whereas the samples from only one farm of the ten considered were found to contain a slightly higher average lead concentration than permitted by the Serbian standard. A lead concentration of 0.86 mg/kg wet weight was detected in one liver sample from this farm. The maximum permitted lead concentration was exceeded in the liver samples obtained from three farms. However, all the liver samples analysed were found to contain the permitted levels of cadmium, with an average cadmium concentration of 0.12 mg/kg wet weight and a maximum cadmium concentration of 0.48 mg/kg wet weight. The occurrence of heavy metals and their origin in the pig’s offals should be examined in greater detail in future research, especially because pig’s offals are used in the meat processing industry.


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