Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:Q9UIJ5 (Rec)
 58,342 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This paper reports adsorptive endocytosis of exogenous proteins by the trophotaenial absorptive cells (TACs) in the viviparous goodeid teleost, Ameca splendens. In vitro incubations were performed with gold conjugated to bovine serum albumin (Au-BSA), human transferrin (Au-HTf), fetuin (Au-Fet), and asialofetuin (Au-ASFet). Localization of gold label on the TAC surface was nearly exclusive to patches of an amorphous coat associated with part of the intermicrovillous plasma membrane. On addition of excess BSA, HTf, Fet, or ASFet to incubation media containing, respectively, Au-BSA, Au-HTf, Au-Fet, or Au-ASFet, the density of gold particles adsorbed on the TAC surface decreased drastically. Moreover, attachment of the four protein-gold complexes to the same plasma membrane sites was suggested by reciprocal inhibitory effects. Further proteins such as hemoglobin, myoglobin, and cytochrome c were as well potent inhibitors of Au-BSA and Au-HTf binding and uptake. Binding of TACs of native BSA or HTf was visualized by immunogold labeling. The interactions between proteins and binding sites required both the presence of Ca2+ and appropriate pH greater than 6.6. Analyses of the concentration-dependent BSA and HTf binding curves, plotted from morphometric data, resulted in apparent dissociation constants, Kds, of approximately 5 x 10(-7) M and 4 x 10(-7) M, respectively. Following binding at the TAC surface and internalization via clathrin-coated pits and vesicles the several ligands were routed along the lysosomal pathway with transit through the endosomal compartment. Prolonged incubation periods led to massive intracellular accumulation of tracer proteins. The effects of NH4Cl (10 mM) treatment on TACs included enormous cytoplasmic vacuolation, a reversible loss of protein binding sites on the plasma membrane, and a block in the transport of protein-gold complexes to lysosomes.
Anat Rec 1992 Jul
PMID:Protein-gold transport in the endocytic complex of trophotaenial absorptive cells in the embryos of a goodeid teleost. 160 71

Recombinant murine interferon-gamma (Rec-MuIFN-gamma) was administered intramuscularly to C3H/HeNCrj mice on Days 6-15 of gestation at dosage levels of 8 X 10(5), 4 X 10(6), and 2 X 10(7) u/kg/day. Dams were killed for examination of fetuses on Day 18 of gestation. Pregnant females that received 2 X 10(7) u/kg/day of Rec-MuIFN-gamma showed uterine bleeding on Days 10-15 of gestation and could not maintain their pregnancy. These dams died on Days 13-17 or were killed in extremis on Days 10-15 for examination, and therefore no fetal data were available for this group. In the 2 X 10(7) u/kg/day group, the mean absolute weights of the lung and spleen increased and the mean absolute weight of the liver, red blood cells (RBC), hematocrit, and hemoglobin decreased significantly. Surviving dams in the 8 X 10(5) and 4 X 10(6) u/kg/day groups showed significant increases in the mean absolute weights of the lung, liver, kidneys, and spleen and a decrease in platelet count. Significant increases in the weights of the heart and ovaries and decreases in RBC, hematocrit, and hemoglobin were observed in the 4 X 10(6) u/kg/day group. Histopathological examination revealed increased extramedullary hematopoiesis in the spleen of the 4 X 10(6) and 2 X 10(7) u/kg/day groups. Fetuses showed no external, visceral, or skeletal malformations and variations caused by the administration of Rec-MuIFN-gamma in any of the treated groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effects of recombinant murine interferon-gamma on pregnant mice and their fetuses. 211 76

Differentiation of circulating erythrocytes was inhibited by all-trans-retinoic acid in 8- and 9-day-old mouse embryos exposed in utero on day 8 of gestation. Histochemical, light, and electron microscopic examinations revealed that all-trans-retinoic acid first decreased the numbers of polychromatic erythroblasts and then increased them. Simultaneously, immature erythroblasts proliferated, and differentiation of mature primitive erythrocytes was suppressed. Electron microscopy of these immature erythroblasts revealed monosomes rather than the usual polyribosomes in the cytoplasm. RNA histochemistry revealed a greater number of intermediate differentiating erythroblasts with pyronin-positive cytoplasm than those in the controls and revealed also a dose-dependent relationship between the number of polychromatic erythroblasts with positive pyronin staining and the controls. These findings suggest that all-trans-retinoic acid destroys the messenger RNA system, resulting in an inability to produce hemoglobin.
Anat Rec 1989 Jan
PMID:Suppression of erythroid cell differentiation in mouse embryos exposed to retinoic acid in utero. 246 57

In 3-day-old embryos the aortic cell clusters formed two parallel ridges in the ventrolateral part of the aorta. The border of the somato- and splanchnopleures close to the aorta showed a very intensive cell proliferation and a cell emigration up to the aorta. This cell flow and the bilateral appearance of the intraaortic ridges suggested that the aortic cell clusters originated from the coelomic epithelium. This intraembryonic hemopoietic stem cell formation from the splanchnopleure was comparable to that of the blood island formation in the yolk sac from extraembryonic splanchnopleure. The appearance of the white blood cells and definitive erythrocytes with adult-type hemoglobin was preceded by the aortic cell clusters. We concluded that the stem cells of the adult-type blood developed from the aortic cell clusters whereas the blood islands of the yolk sac may contribute only the primitive red blood cells.
Anat Rec 1988 Sep
PMID:Origin of aortic cell clusters in the chicken embryo. 318 88

An attempt has been made to assess the importance of systemic sites of interaction from the effect of dietary molybdenum (Mo) on the protection afforded by a single sc injection of copper (Cu) to 29 initially hypocupraemic 5-year-old ewes, maintained on a low Cu diet. They were fed a diet of 1 kg/day containing 1.3 mg of Cu/kg supplemented with sodium sulphate which provided 1.7 gm of sodium per kg. Group A was given no further supplement. Group B was given added Mo, 25 mg/kg. Group C was given added Cu, 10 mg/kg. After 7 months, several animals in each group were injected sc with a single dose of 46.5 mg of Cu in the form of copper calcium edetate (Coprin). Blood samples were taken at intervals from the injected ewes over a 250-day period. All ewes were mated after 12 months on the diet. Injected ewes were approaching the 4th month of gestation when the last blood sample was taken at 250 days. Total Cu in plasma was determined by atomic absorption spectometry. Direct reacting Cu in plasma, cerulosplasmin oxidase activity, and hemoglobin were also estimated. Plasma Cu concentrations had increased to normal levels in 14 days in Group A after the Cu injections. Group B animals showed a greater increase, mean values exceeding those of Groups A and C, between Days 28-129 (p less than .01). Plasma Cu levels declined in ewes not given supplementary Cu after the 177th day. The final values for Groups A and B were similar to those found before injection. The direct reacting Cu in each group was increased after 7 days (p less than .05). This effect was most marked in the Mo supplemented ewes (Group B). The effect of Mo persisted until the final bleeding. Direct reacting Cu was only a minor part of the early response in total plasma copper of Group B ewes. Dietary Mo did not inhibit the incorporation of injected Cu into ceruloplasmin. The Mo-supplemented ewes were in poorer condition than copper-supplemented ewes. All groups gained in weight after the injections. The sc injection of Cu at 5 months prior to mating imporved fertility in Groups A and B. There was no evidence that dietary Mo impa ired the metabolism of parenteral Cu. However, it is known to deplete r uminants of Cu when the diet provides the only source of Cu. It is ther efore thought that the site of the Cu with Mo interaction is in the gut. If infertility due to Cu deficiency is suspected in a flock, an injection of Cu immediately prior to mating may improve conception rate and provide sufficient Cu to reduce the incidence of swayback.
Vet Rec 1974 Aug 24
PMID:The effect of dietary molybdenum on hypocupraemic ewes treated by subcutaneous copper. 444 11

A description of the efficient high-level expression of the monomer hemoglobin (GMG4) from Glycera dibranchiata is presented. The cDNA described by Simons and Satterlee [Simons, P.C., & Satterlee, J.D. (1989) Biochemistry 28, 8525-8530] was subcloned into an expression system, and conditions were found that led to the production of large amounts of soluble apoprotein (rec-gmg). These conditions included lowering the temperature during the induction period and growth in a rich medium with a higher ionic strength. Characterization of this reconstituted recombinant protein showed that it was not identical to the native GMH4 protein. Both UV-visible and 1H NMR data indicated differences within the holoprotein (rec-gmh) heme pocket compared to the native protein, the major difference being that two nonidentical heme orientations are significantly populated in rec-gmh. This phenomenon has been seen previously in other heme proteins, where these heme orientational isomers are described by a 180-deg rotation about the heme alpha-gamma meso axis. This work prompted the production of a complete chemical sequence for the native GMH4 [Alam S.L., Satterlee, J. D., & Edmonds, C. G. (1994) J. Protein Chem. 13, 151-164], which showed that the expressed rec-gmg protein differed at three primary sequence positions (41, 95, and 123) from the native component IV globin (GMG4). Subsequently, we have produced the triple-revertant mutations required to express the recombinant wild-type protein (recGMG4). The physical characteristics of the active site in the holoprotein (recGMH4) are identical to those of the native protein.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Expression of recombinant monomer hemoglobins (component IV) from the marine annelid Glycera dibranchiata: evidence for primary sequence positional regulation of heme rotational disorder. 806 70

A study was conducted among children under 12 years of age admitted to a rural district hospital in western Kenya to evaluate the use of blood transfusions, identify risk factors for severe anemia, and determine when transfusion improves survival of severely anemic children. A total of 2433 children were admitted to the pediatric ward during the 12-month study period; 29% (684) had severe anemia (hemoglobin [Hb] 5.0 g/dl) and 20% (483) received blood transfusions. Among children admitted with Hb 5.0 g/dl, 19% (124/663) had a history of prior transfusion, compared with only 6% (94/1607) with Hb 5.0 g/dl. The fatality rate of all children with Hb 5.0 g/dl was 18%, compared with 8% among children with Hb 5.0 g/dl. Among all children who died during hospitalization, 48% had a Hb 5.0 g/dl at the time of admission. Children younger than 3 years old accounted for 92% of admissions with Hb 5.0 g/dl, 87% of all pediatric deaths, and 92% of all pediatric transfusions. Age under 3 years and malaria parasitemia were associated with severe anemia. 88% (424) of transfusions were given to children with Hb 5.0 g/dl. Only 25% (120) of transfused children received blood on the date of admission, while 34% (161) were transfused the day after admission, and 41% (199) were transfused 2 or more days after admission. Among children with Hb 5.0 g/dl, 40% (274/683) were not transfused. If transfusions had been given only within the first 2 days of admission to children with respiratory distress and Hb 5.0 g/dl, the frequency of transfusion would have been reduced by 55% without increasing mortality. Prevention and effective treatment of the causes of anemia (such as malaria), targeted to children under 3 years of age, are critically needed to limit pediatric anemia, blood transfusion, and anemia-associated mortality.
Wkly Epidemiol Rec 1994 Mar 11
PMID:Global programme on AIDS. A study on the effect of blood transfusion on survival among children in a Kenyan hospital. 819 7

Erythropoietin (Epo) is the central regulator of red blood cell production and acts primarily by inducing proliferation and differentiation of erythroid progenitor cells. Because a sufficient supply of iron is a prerequisite for erythroid proliferation and hemoglobin synthesis, we have investigated whether Epo can regulate cellular iron metabolism. We present here a novel biologic function of Epo, namely as a potential modulator of cellular iron homeostasis. We show that, in human (K562) and murine erythroleukemic cells (MEL), Epo enhances the binding affinity of iron-regulatory protein (IRP)-1, the central regulator of cellular iron metabolism, to specific RNA stem-loop structures, known as iron-responsive elements (IREs). Activation of IRP-1 by Epo is associated with a marked increase in transferrin receptor (trf-rec) mRNA levels in K562 and MEL, enhanced cell surface expression of trf-recs, and increased uptake of iron into cells. These findings are in agreement with the well-established mechanism whereby high-affinity binding of IRPs to IREs stabilizes trf-rec mRNA by protecting it from degradation by a specific RNase. The effects of Epo on IRE-binding of IRPs were not observed in human myelomonocytic cells (THP-1), which indicates that this response to Epo is not a general mechanism observed in all cells but is likely to be erythroid-specific. Our results provide evidence for a direct functional connection between Epo biology and iron metabolism by which Epo increases iron uptake into erythroid progenitor cells via posttranscriptional induction of trf-rec expression. Our data suggest that sequential administration of Epo and iron might improve the response to Epo therapy in some anemias.
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PMID:Regulation of cellular iron metabolism by erythropoietin: activation of iron-regulatory protein and upregulation of transferrin receptor expression in erythroid cells. 900 72

The aim of the present study was to investigate whether the breathing of hyperoxic gas affects hemoglobin oxygen saturation (S(a)O(2)) and blood acidosis during intense intermittent exercise and recovery in sprint runners. The hypothesis was that the breathing of hyperoxic gas prevents S(a)O(2) from decreasing, delays blood acidosis during the exercise and improves the rate of heart rate recovery after the exercise. Nine sprinters ran three sets of 300 m at different velocities on a treadmill in normoxia (NOX) and in two hyperoxic conditions (ERHOX and RHOX; F(I)O(2) 0.40) in a randomized order. In ERHOX the inspired air was hyperoxic during the entire exercise and recovery and in RHOX the hyperoxic air was only inhaled during recovery periods. Blood pH and S(a)O(2) were measured from fingertip blood samples taken after each set of runs. The mean heart rate for the final 15 s of the last run in each set (HR(work)), the mean heart rate for the final 15 s of the first minute of recovery (HR(rec)) and the difference of HR(work) and HR(rec) (HR(dec)) were determined. In NOX, S(a)O(2) decreased from 95.0 +/- 2.0% to 88.7 +/- 2.0% (p < 0.001) but S(a)O(2) did not change in ERHOX (from 95.4 +/- 1.3% to 95.9 +/- 1.8%). A significant correlation was observed between the S(a)O(2) decrease in NOX and the effect of hyperoxia on blood pH in ERHOX (r = 0.63) and on HRdec in both ERHOX (r = 0.74) and RHOX (r = 0.69). We concluded that hemoglobin oxygen de-saturation occurred during intensive intermittent exercise in normoxia but hyperoxic gas during the exercise prevents S(a)O(2) from decreasing. Furthermore, the present results suggested that the beneficial effects of hyperoxia on heart rate recovery and blood acidosis during intensive intermittent exercise were related to hemoglobin de-saturation in normoxia.
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PMID:Effect of hyperoxia on metabolic responses and recovery in intermittent exercise. 1238 77

Gaucher disease (GD) is an autosomal recessive inborn error of metabolism, resulting from a deficiency of the enzyme glucocerebrosidase, causing an accumulation of the glycolipid glucocerebroside within lysosomes of macrophages in the reticuloendothelial system. Three major clinical forms have been assigned and more than 200 gene mutations have been identified. We herein report a Lebanese boy born with a novel combined mutation L371V/Rec-NciI, who presented with moderate-severe type 1 GD. An overview of the clinical and biomarker improvement following enzyme replacement therapy with imiglucerase is described in a follow-up of 30 months. Imiglucerase seems to be efficacious in decreasing the severity of the disease associated with this mutation. However, a high dose may be required to achieve optimal growth, platelet count, and hemoglobin level.
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PMID:A novel genotype c.1228C>G/c.1448C-1498C (L371V/Rec-NciI) in a 3-year-old child with type 1 Gaucher disease. 1902 90


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