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PubMed1987

Pharmacologic amino acid acylation in the acute hyperammonemia of propionic acidemia.

An infant with propionic acidemia presented at one month of age with hyperammonemic coma. Treatment by two double-volume exchange transfusions did not have an appreciable effect, but hemodialysis led to a substantial reduction of the serum concentration of ammonia on two occasions. Nevertheless, continued therapy with sodium benzoate, arginine-HCl, carnitine and lactulose did not have any observable effect on the blood concentration of ammonia. Treatment with sodium phenylacetate was followed by a reduction in serum concentrations of ammonia to normal levels which were maintained. These observations demonstrate the dramatic reduction in serum concentrations of ammonia that may be obtained in patients with organic acidemia by hemodialysis. They suggest that pharmacologic acylation therapy with phenylacetate may be of lasting benefit in the management of this complication.

Acylation↗
PubMed1986

[Azoles. 17. Beta-(4-pyrazol)acrylic and propionic acids and their anti-inflammatory activity].

beta-(4-Pyrazole)acrylic acids 22-28 were prepared by the Knoevenagel reaction of malonic acid and 4-formylpyrazoles 8-14. 4-Pyrazolemethylenemalonic acids 15-21 were isolated as intermediates. The latter compounds were also synthesized by treating the 4-formylpyrazoles 8-14 with diethyl malonate followed by hydrolysis of the obtained diethyl esters 15a-21a. The effect of piperidine and pyridine on the Knoevenagel condensation was investigated. The beta-(4-pyrazole)acrylic acids 22-27, on catalytic reduction, gave the corresponding beta-(4-pyrazole)propionic acids 29-34. Compounds 23, 24, 27, 29-31 and 34 appeared to be less active than phenylbutazone in carrageenin-induced oedema test, but they were less toxic than the reference drug.

Acrylates↗
PubMed1979

Prenatal diagnosis of propionic acidemia.

Prenatal diagnosis of a fetus with propionic acidemia has been accomplished by the detection of methylcitrate, a unique metabolite, in the amniotic fluid by liquid partition chromatography and gas chromatography-mass spectrometry. The diagnosis was confirmed by demonstration of deficient activity of propionyl-CoA carboxylase in cells cultured from the amniotic fluid and in fetal tissues. In two subsequent pregnancies, methylcitrate was not present in amniotic fluid. Enzyme assay indicated that one fetus was heterozygous and the other was normal, and healthy infants were born at term. The analysis of organic acids in amniotic fluid permits very rapid prenatal diagnosis, usually within 48 hours after obtaining the fluid. It may provide a general method in conditions in which an unusual metabolite can be identified.

Abortion, Therapeutic↗
PubMed1980

[Propionic acidemia associated with hypertrophic pyloric stenosis and bouts of severe hyperglycemia (author's transl)].

A newborn is presented with hyperexcitability, drowsiness and later-on with frequent vomiting and muscular hypotonia. Examination of the urine by gas chromatography-mass spectrometry lead to the diagnosis of propionic acidemia which was confirmed enzymatically in fibroblasts. Two unusual features were encountered in this case: There were severe bouts of hyperglycemia with blood glucose values up to 396 and 747 mg/100 ml; furthermore x-ray studies and autopsy revealed a hypertrophic pyloric stenosis.

Autopsy↗
PubMed1981

Propionic acid-metabisulphite-Schiff reagent for quick Feulgen staining.

This communication presents a method for the preparation of Schiff reagent in which N hydrochloric acid has been replaced by a low concentration of propionic acid. The result of using such a Schiff reagent indicates that the dye-reagent thus prepared is extrafast in action on acid-hydrolysed mammalian tissue sections. The intensity of nuclear colouration is also increased considerably, since the pH of the dye-reagent is 6.0. It is, therefore advocated that this newly developed Schiff reagent be used at 5 degrees C.

Animals↗
PubMedDate not supplied

Cerebral CT findings in methylmalonic acid propionic acidemias.

The cerebral computed tomographic findings in two infants with methylmalonic acidemia and one infant and one adult with propionic acidemia are presented. Pertinent metabolic, clinical, and pathologic features of these genetic disorders of vitamin B12 (cobalamin) and biotin metabolism are reviewed briefly. Computed tomographic abnormalities consist of focal or diffuse deep cerebral hemisphere lucencies and diffuse loss of brain substance. These findings correlated well with the clinical and pathologic findings. The leukoencephalopathy in these uncommon autosomal recessive conditions appears to be due to ketoacidosis, which in some cases may be managed by vitamin supplementation.

Adolescent↗
PubMed1980

Pharmacological properties of a new anti-inflammatory agent: 2-(2-isopropyl-5-indanyl)propionic acid (UP 517-03).

2-(2-Isopropyl-5-indanyl)propionic acid (UP 517-03) is a new anti-inflammatory agent with marked analgesic and antipyretic properties. The anti-inflammatory potency is greater than that of phenylbutazone but lower than that of indometacin. This effect is observed also in adrenalectomized rats. The analgesic activity in the Randall and Selitto test is equipotent to that of indometacin, but this potency is lower in the other tests. In lowering brewer's yeast-induced fever in rats, UP 517-03 is as potent as indometacin and 10 times more effective than phenylbutazone. Considering from pharmacological index, UP 517-03 is tolerated as well as phenylbutazone and better than indometacin.

Analgesics↗
PubMed1981

Screening system for the evaluation of preparations with potential cytostatic and immunotropic activity. II. Derivatives of propionic and hydroxybutyric acids and of tetrahydrofuranone-2.

New screening system was used for the evaluation of preparations wih potential anti-mitotic, cytostatic and secondarily, immunodepressive activity. A group of compounds, derivatives of propionic and hydroxybutyric acids and of tetrahydrofuranone-2 was analyzed. It was showed that among them there were preparations reducing 14C-thymidine incorporation to lymphocyte nuclei. The authors pointed to the fact that in this mechanism, beside specific component, nonspecific component takes part, which results from the change of pH of the medium by the solutions of the investigated preparations. Relation between chemical structure and pharmacological activity in this group of compounds is discussed.

Antineoplastic Agents↗
PubMed1980

[The mechanism of anti-vascular permeability of 2-(2-fluoro-4-biphenylyl) propionic acid(Flurbiprofen) (author's transl)].

Effect of 2-(2-fluoro-4-biphenylyl) propionic acid(Flurbiprofen, FP-70) on increased vascular permeability was studied using the potentiation of bradykinin-induced vascular permeability by prostaglandin in the guinea pig skin, as the test system. Prostaglandin E1 or E2 (1 microgram/site) alone did not show increased vascular permeability but did enhance the effect of various concentrations of bradykinin when concomitantly applied, whereas prostaglandin F2 alpha did not potentiate the effect of bradykinin on vascular permeability. Arachidonic acid (100 microgram/site) had no effect on vascular permeability when injected alone or in combination with bradykinin. However, arachidonic acid enhanced the effect of bradykinin when given 30 min before bradykinin. FP-70 had no inhibitory effect on prostaglandin E1 potentiation of bradykinin-induced vascular permeability. On the other hand, FP-70 completely abolished the enhancement of the effect of bradykinin by arachidonic acid. These results strongly suggest that anti-vascular permeability action of FP-70 may be due to inhibition of prostaglandin synthetase. Indomethacin and acetylsalicylic acid also exhibit similar effects. However, the inhibitory effect of FP-70 was 12.5 and 166.7 times as potent as indomethacin and acetylsalicylic acid, respectively.

Animals↗
PubMed1980

Genetics of propionic acidemia in a Mennonite-Amish kindred.

A large Mennonite kindred was found to have propionic acidemia (complementation group pcc C) in at least four different sibships. Even within this kindred and this complementation group (where etiology may be assumed to be identical), there is a wide range of symptoms exhibited by homozygous pcc C-deficient individuals. The inbreeding coefficients (f) for the affected sibships ranged from 4.776 X 10(3) to 2.003 X 10(-2). Data from this study strongly support the single-locus autosomal recessive mode of inheritance. Three couples were found to be common in the ancestry (9--11 generations ago) of all eight parents of the four affected sibships. Relative likelihoods for a member of each of those couples to have been the early carrier of the defective allele were calculated at 1539, 278, and 1. Thus, one couple was designated the most likely earliest-known transmitter of the pcc-deficient allele.

Adult↗
PubMed1995

Nonsteroidal antiinflammatory agents. XXIII. Synthesis and activity of stereomeric (+/-)-erythro- and (+/-)-threo-2-(4-biphenylyl)-3-hydroxy-2-methyl-3-phenyl-propionic acids.

In pursuing our research on the NSAIDs, the diastereomeric (+/-)-erythro- and (+/-)-threo-2-(4-biphenylyl)-3-hydroxy-2-methyl-3-phenyl-propionic acids (4 and 5) were synthesized; the last was also resolved in its optical isomers (6 and 7). The attribution of the relative configuration to 4 and 5 was performed by means of X-ray analysis. The compounds were tested for their antiinflammatory activity. The acid 4, which resulted very interesting, was tested also for analgesic and antipyretic activity, as well as for behavioural effects, gastric tolerability and acute toxicity. On the basis of the obtained data, this compound resulted a very promising one.

Animals↗
PubMedDate not supplied

Propionic acidemia without acidemia: a case report.

Propionic acidemia is a recessively inherited disorder of organic acid metabolism characterized by a spectrum of clinical and biochemical findings. Metabolic acidosis is a key feature of this disease and is useful in differentiating it from nonketotic hyperglycinemia. This case report is presented because significant metabolic acidosis was not a prominent or persistent finding in this patient.

Acidosis↗
PubMed1994

Mutations participating in interallelic complementation in propionic acidemia.

Deficiency of propionyl-CoA carboxylase (PCC; alpha 4 beta 4) results in the rare, autosomal recessive disease propionic acidemia. Cell fusion experiments have revealed two complementation groups, pccA and pccB, corresponding to defects of the PCCA (alpha-subunit) and PCCB (beta-subunit) genes, respectively. The pccBCC group includes subgroups, pccB and pccC, which are thought to reflect interallelic complementation between certain mutations of the PCCB gene. In this study, we have identified the mutations in two pccB, one pccC, and two pccBC cell lines and have deduced those alleles participating in interallelic complementation. One pccB line was a compound heterozygote of Pro228Leu and Asn536Asp. The latter mutation was also detected in a noncomplementing pccBC line. This leaves Pro228Leu responsible for complementation in the pccB cells. The second pccB line contained an insertional duplication, dupKICK140-143, and a splice mutation IVS + 1 G-->T, located after Lys466. We suggest that the dupKICK mutation is the complementing allele, since the second allele is incompatible with normal splicing. The pccC line studied was homozygous for Arg410Trp, which is necessarily the complementing allele in that line. For a second pccC line, we previously had proposed that delta Ile408 was the complementing allele. We now show that its second allele, "Ins.Del," a 14-bp deletion replaced by a 12-bp insertion beginning at codon 407, fails to complement in homozygous form. We conclude that the interallelic complementation results from mutations in domains that can interact between beta-subunits in the PCC heteromer to restore enzymatic function. On the basis of sequence homology with the Propionibacterium shermanii transcarboxylase 12S subunit, we suggest that the pccC domain, defined by Ile408 and Arg410, may involve the propionyl-CoA binding site.

Alleles↗
PubMedDate not supplied

[Antimutagenic effect of culture fluid obtained as a result of propionic acid fermentation].

It was shown for first time that propionic acid bacteria excrete in the medium substances with antimutagenic activity against mutagenicity of 4-nitroquinoline-N-oxide in Salmonella typhimurium TA100. The substances act as antimutagens. Antimutagenic action of filtrate of culture liquid increased with time preincubation with mutagen and dose increase. The most activity was found in the culture of logarithmic and early stationary phase (24-48 h). The substances with antimutagenic activity are formed in the medium with glucose, lactose and lactate. Antimutagenic factor (factors) is dialysable, thermostable and is not presented with peptide.

4-Nitroquinoline-1-oxide↗
PubMed1996

Carmine-propionic acid stain for elucidation of fine cellular structure in nematodes.

Carmine-propionic acid staining was found to enhance the elucidation of cellular membranes in the esophagus and genital tract and the structure of sperm in strongylate nematodes. This regressive staining technique may also have general utility for differentiation of cellular membranes and nuclear structure in other taxa of parasitic helminths.

Animals↗
PubMed1996

Synthesis of indole-N-propionitrile, N-propionic acid and N-propionamide derivatives of potential biological activity.

Cyanoethylation of a selected group from indole derivatives 1a-i using acrylonitrile in pyridine and potassium hydroxide afforded the corresponding indole-1-propionitrile 2a-h derivatives which readily hydrolysed in basic medium to the corresponding indole-1-propionic acid derivatives 4a-c. A several attempts to cyclize the above products-to the corresponding dihydropyrroloquinolin-4-one derivatives. The indole-1-propionamide derivatives reacted with diethyl carbonate and ethyl acetate under Claisen condensation also, with diazonium salts and nitric acid.

Amides↗
PubMedDate not supplied

A YAC contig spanning the blepharophimosis-ptosis-epicanthus inversus syndrome and propionic acidemia loci.

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is an autosomal dominant condition consisting of congenital dysplasia of the eyelids with a reduced horizontal diameter of the palpebral fissures, droopy eyelids and epicanthus inversus. Two clinical entities have been described: type I and type II. The former is distinguished by female infertility, whereas the latter presents without other symptoms. Both type I and type II were recently mapped on the long arm of chromosome 3 (3q22-q23), suggesting a common gene may be affected. The centromeric and the telomeric limits of this region are well defined between loci D3S1316 and D3S1615, which reside approximately 5 cM apart. Here, we present the construction of a YAC contig spanning the entire BPES locus using 17 polymorphic markers, 2 STS and 28 ESTs. This region of approximately 5 Mb was covered by 31 YACs, and was supported by detailed FISH analysis. In addition, we have precisely mapped the propionyl-CoA carboxylase beta polypeptide (PCCB), the gene mutated in propionic acidemia, within this contig. Apart from providing a framework for the identification of the BPES gene, this contig will also be useful for the future identification of defects and genes mapped to this region, and for developing template resources for genomic sequencing.

Amino Acid Metabolism, Inborn Errors↗