Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:6.3.5.5 (CPS)
 1,262 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The effects of corticosteroids and pancreatic hormones on two mitochondrial enzymes of ureagenesis, carbamyl phosphate synthetase-I (CPS-I) and ornithine transcarbamylase (OTC), were investigated and compared in fetal rat liver. Supplementing hydrocortisone acetate (50 micrograms) to 18.5-day-old fetuses significantly increased CPS-I activity (by 36%) and decreased OTC activity (by 23%). An actinomycin D supply (2 micrograms) to 18.5-day-old fetuses prematurely increased OTC activity and decreased fetal insulin level (by 42%). This treatment had no effect on CPS-I activity. Glucagon supply (25 micrograms) during the late fetal period increased both activities within 2 h, while dibutyryl-cAMP enhanced OTC activity 17 h later. These results suggested that the fetal development of CPS-I activity was under the control of corticosteroids and glucagon. In contrast, corticosteroid hormones produced an inhibitory effect on OTC activity. This might be explained by the permissive effect of corticosteroids on insulin action, since insulin might act as a repressor in utero of enzyme development. Thus, the paradoxical effect of actinomycin D on OTC activity was probably due to the decrease in fetal insulinemia.
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PMID:Effects of corticosteroids and pancreatic hormones on carbamyl phosphate synthetase-I and ornithine transcarbamylase activities in fetal rat liver. 299 95

We have partially characterized an intracellular fraction from Phycomyces blakesleeanus which shows proteolytic activity. The apparent thermal inactivation constant (Kd) was 0.12 min-1 at 50 degrees C. This proteolytic fraction was split into two active fractions by ultrafiltration using a membrane with an exclusion size of 30,000. Both fractions were inhibited by phenyl methyl sulphonyl fluoride. The Ki value for the fraction with molecular weight greater than 30,000 was 0.075 mM. The fraction with molecular weight less than 30,000 inactivated the Phycomyces CPS.
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PMID:Partial characterization of intracellular protease activity which participates in the inactivation of carbamyl phosphate synthase in Phycomyces blakesleeanus. 306 66

A partial carbamylphosphate synthetase (CPS: EC 6.3.4.16) deficiency (McKusick 23730) was found in a male child who presented with generalized convulsions, rickets and apnoeic attacks at six months of age. By his second year he showed serious developmental delay and a gut biopsy revealed an absence of CPS activity with an elevated ornithine transcarbamylase activity. Analysis of the gut biopsy sample on SDS-polyacrylamide gels, followed by electrophoretic transfer to a nitrocellulose filter probed with monospecific antibodies to CPS showed that the child had normal levels of immunoreactive enzyme, but instead of one band corresponding to normal CPS with a subunit size of 165,000 u, the patient had three immunoreactive bands, one larger and two smaller than that found in normal controls. The genetic defect in this child therefore results in an unusual form of CPS being made which has markedly reduced enzyme activity.
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PMID:Immunological evidence for a carbamylphosphate synthetase lesion resulting in the formation of enzyme with altered sub-unit size. 310 74

The ADP-ribosylation factor (ARF) is a 21-kDa GTP-binding protein that serves as the cofactor in the cholera toxin-catalyzed activation of the stimulatory guanine nucleotide-binding protein of adenylate cyclase (Gs). An oligonucleotide probe based on the partial amino acid sequence was used to clone ARF from a bovine adrenal chromaffin cDNA library. The yeast (Saccharomyces cerevisiae) ARF gene was then cloned from a YCp50 genomic library by cross-species hybridization by using the coding region of the bovine gene. RNA gel blots of poly(A)+ RNA indicate that only one ARF message size (900 and 2000 base pairs) is present in yeast and cows, respectively. Comparison of the cDNA-derived amino acid sequences of ARF to other GTP-binding proteins reveals a structural relationship between ARF and the ras family of proteins. A slightly better structural relationship is detected when ARF is compared to the alpha subunits of the trimeric GTP-binding proteins, including Gs alpha. All of the biochemical characteristics of the purified ARF, including the lack of GTPase activity and the posttranslational myristoylation, are consistent with the derived sequences. Comparison of the ARF sequences to that of the chicken processed pseudogene (CPS-1), previously reported as a ras homologue, reveals that CPS-1 is actually an ARF-derived gene. These results demonstrate that ARF is a GTP-binding protein with structural features of both the ras and the trimeric GTP-binding protein families.
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PMID:Sequences of the bovine and yeast ADP-ribosylation factor and comparison to other GTP-binding proteins. 313 54

Plasma fibronectin (FN) has been demonstrated to serve as an opsonin involved in the ingestion of foreign particles by phagocytes. This study concerns the effect of FN exposure on the respiratory burst of normal human peripheral phagocytes, using a luminol-dependent chemiluminescence (CL) assay for measurement of reactive oxygen metabolites generated. FN enhanced, in a dose-dependent manner, the CL response of circulating monocytes stimulated, probably via beta-glucan receptor, with unopsonized zymosan. FN also increased the CL response of phagocytes to fresh serum-opsonized zymosan. When we used a glycolipid (ceramide pentasaccharide, CPS) incorporated on liposome membranes as an antigen, the immune complexes prepared between CPS and human IgG (as antibody) did not induce a CL response, differing from previous reports. Addition of FN to the immune complexes significantly enhanced the CL response of phagocytes. The role of FN in host defence is discussed.
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PMID:Fibronectin enhances respiratory burst of phagocytes stimulated by zymosan and immune complexes. 319 70

The data emerging from our study are the following: the presence of an identifiable cause is important: complications like tuberous sclerosis or signs of marked cerebral damage represent an adverse risk factor for IE. The presence of epilepsy among relatives, evidence of pre- or perinatal cerebral damage, mental retardation, and early onset, long periods of uncontrolled seizures before starting an adequate therapy and frequency of seizures appear to be indicative of an adverse prognosis, since differences between the two groups of responsive or unresponsive patients are statistically significant. On the contrary, the occurrence of febrile convulsions in the past history does not seem to have an adverse prognosis. Temporal lobe epilepsy and IS bear the worst prognosis. ME, CPS, GTCS, SPS, LGS and PM have a progressively better outcome in responsiveness to AEDs. Concerning therapy in patients with IE, studies indicate the results of high dose monotherapy appear to be equal or better than with polypharmacy. Because of the gravity of the situation, trials with unconventional drugs have been performed, but it is too early to draw definite conclusions about the long-term usefulness of most of them. In conclusion, our data indicate that the appearance of an IE can be predicted utilizing the above mentioned criteria, considered either alone or in combination. The issue of IE remains undoubtedly an important one among the group of convulsive disorders. Further studies considering a greater number of patients and new therpeutic strategies are to be recommended.
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PMID:Intractable epilepsy: etiology, risk factors and treatment. 329 46

In adults Hib CPS protein conjugates are much more immunogenic than the polysaccharide alone; further studies have shown that they induce a booster response in children. The antibodies produced in response to the conjugates have the same biologic properties, isotype and IgG subclass composition as those elicited by Hib CPS alone or those present in serum after convalescence from Hib disease. More recently attempts have been made to make the conjugates compatible with DTP vaccine. In this procedure DTP is absorbed onto aluminum compounds (aluminum hydroxide or phosphate), with the effect of significantly prolonging diphtheria and tetanus antibody synthesis. Adsorption of the Hib CPS conjugate under controlled conditions does not alter the total amount of antibody elicited in infant rhesus monkeys after the third or final injection. The appearance of Hib CPS antibodies after the first injection, however, is accelerated with conjugate that has been adsorbed. This is an encouraging finding, because it means that more polysaccharide conjugates can be compatible with existing DTP vaccine.
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PMID:Haemophilus influenzae type b: the search for a vaccine. 331 43

Plasma filtration characteristics of three hollow fiber plasma separation filters (Plasmaflo Hi-05, Extraplex BL 550 and CPS-10) were studied in a single-needle setting by means of the double headpump. Plasma exchange was carried out in 12 patients during a total number of 59 sessions. For each filter, a mean total ultrafiltration volume of +/- 3,000 ml was obtained over a period from 92 to 121 min. The lowest and highest obtained mean filtration flows were 26.7 +/- 2.5 and 36.6 +/- 1.7 ml/min for Extraplex BL 550 and CPS-10, respectively (p less than 0.01). The pre- and postplasmapheresis pressures, measured in the bubble trap chamber as an indirect estimation of transmembrane pressure, were lower for the Plasmaflo Hi-05 than for the two other filters under study; pressures remained unaltered during the session. Blood pressure showed a minor but significant decline during plasmapheresis with the Plasmaflo filter. A reduction after plasmapheresis by more than 40% of the immunoglobulins IgE, IgG, IgM and IgA, and of complement factors C3 and C4 was seen for each of the filters and no significant differences between the filters were observed. An additional study on 6 filters with constant blood flow and TMP showed minor differences in the transmembrane pressure necessary to obtain a given filtration volume per unit of time and similar sieving coefficients for immunoglobulins. This study demonstrates that with this single-needle technique a satisfying immunoglobulin extraction performance was obtained for each of the filter types studied; however, there existed minor but significant differences in the patient hemodynamic status according to the membrane used.
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PMID:Single-needle membrane plasmapheresis. In vivo comparison of plasma separator performances. 339 73

Clinical features, ictal manifestations, EEG and CT findings of two patients with CPS are presented. Status consisted of confusion associated with continuous focal EEG findings in both cases.
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PMID:Complex partial status epilepticus. 341 2

We investigated the distribution of the nuclear encoded mitochondrial enzymes, carbamylphosphate synthetase (CPS; EC 6.3.4.16) and ornithine transcarbamylase (OTC; EC 2.1.3.3) in liver by immunocytochemistry on ultrathin sections using the protein A-gold technique. Both enzymes were found to be present as aggregates in the cytoplasm of hepatocytes, in association with ER membranes adjacent to mitochondria. Clusters of the enzymes were also found inside the mitochondria. The aggregation of these enzymes was found only with antibodies to CPS and OTC and not with antibodies against albumin or with IgG from unimmunized serum, nor were aggregates found in cells other than hepatocytes. The results are suggestive of localized uptake of clusters of enzyme or co-translational uptake of enzyme at discrete localizations and that endoplasmic reticulum (ER) associations may be necessary for uptake of the percursor forms of CPS and OTC. The possible involvement is discussed of micropinosomes which are seen associated with inner membrane, intermembrane space and outer membrane in mitochondria obtained from a perinuclear pellet where ER and mitochondria are frequently found in close association.
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PMID:Import of carbamylphosphate synthetase and ornithine transcarbamylase into mitochondria of rat liver: detection of aggregates of enzyme in cytoplasm and mitochondria using immunoelectron microscopy with the protein A-gold method. 352 29


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