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PubMed1998

[Fluticasone propionate, corticosteroid for topical treatment].

The aim of this study was to evaluate the usefulness of fluticasone propionate in various dermatoses and to present the data to non-dermatologists. The study was performed at the Department of Dermatology, University School of Medicine in Poznań. The studied group consisted of 40 cases of various types of eczema, psoriasis vulgaris, DLE, photodermatoses and morphea. Fluticasone propionate cream or ointment was applied to lesional skin twice daily for 7-19 days. Acute eczematous skin lesions significantly improved after the first seven days of treatment, whereas in patients with chronic eczema the good therapeutic results were observed after 14-19 days. Fluticasone propionate was also effective in the treatment of DLE and photodermatoses.

Administration, Topical↗
PubMed1998

Lichen sclerosus. Therapy with clobetasol propionate.

OBJECTIVE: To assess the efficacy of treating lichen sclerosus with clobetasol propionate. STUDY DESIGN: A retrospective chart review of 81 consecutive symptomatic private practice and clinic patients with biopsy-proven lichen sclerosus were included. All subjects' punch biopsies, baseline histories and physical examinations were reviewed by the same examiner. Each subject's symptomatology and responses to previous treatment modalities were recorded. A standard regimen of 0.05% clobetasol propionate cream was initiated. Subjects were reevaluated at three months and asked to rate the improvement of symptoms. Follow-up examinations were conducted 6-12 months later on 36 subjects. Descriptive statistics and chi 2 analyses were performed. RESULTS: The mean age of subjects was 54 +/- 15.5 years (range, 15-86), and the average duration of treatment prior to clobetasol use was 6 +/- 6.9 years (range, 0.5-29). Twenty-seven subjects did not complete the study or were lost to follow-up. The average subject had tried 2.25 treatment modalities (range, 1-13). The most common symptoms were pruritus (98%) and irritation (61%), with complaints of burning and dyspareunia. Most subjects (76%) had labial involvement, with concomitant involvement of the clitoris (70%), perineum (68%) and perianus (32%). The majority (88%) of subjects had a primary lesion of white and crinkled tissue. With clobetasol, 77% of subjects had complete remission of symptoms, 18% had partial remission and 5% reported no change. A change in clinical appearance was noted for the complete-remission (32%) and partial-remission groups (46%). Twenty-two percent revealed no change. CONCLUSION: Clobetasol propionate cream is recommended for treatment of lichen sclerosus, with a 77% chance of complete remission of symptoms and a 47% chance of improvement in the clinical appearance of the vulva. Women may have to continue to use clobetasol as needed after finishing a base treatment course.

Administration, Topical↗
PubMed2004

Living-donor liver transplantation for propionic acidaemia.

Three patients with the severe form of propionic acidaemia were treated with living-donor liver transplantation (LDLT). The procedure was successful for all patients and the incidence of metabolic decompensation was reduced dramatically even without protein restriction. Biochemically, however, the improvement was not significant and the patients continued to excrete large amounts of propionic acid metabolites. One of the patients experienced a severe acidaemic episode 3 years after transplantation. LDLT has a beneficial effect on the care of severely affected patients since it reduces the risk of metabolic decompensation and improves the quality of life with less strict dietary control. Adequate protein restriction and medication need to be maintained even after successful transplantation.

Amino Acid Metabolism, Inborn Errors↗
PubMed1977

Propionic acidemia and anorectal anomalies in three siblings.

Three siblings suffering from recurrent vomiting, hypotonia, hyperpnea, dehydration, and ketoacidosis were diagnosed as having ketotic hyperglycinemia secondary to propionic acidemia. They also had leukopenia and thrombocytopenia, and two of them had anorectal malformations, one an imperforate anus with rectoperineal fistula, and the other an ectopic anus. The occurrence of propionic acidemia and anorectal anomalies in three siblings out of eight children in a consanguineous marriage suggests an autosomal recessive genetic inheritance.

Amino Acid Metabolism, Inborn Errors↗
PubMed1999

Overview of mutations in the PCCA and PCCB genes causing propionic acidemia.

Propionic acidemia is an inborn error of metabolism caused by a deficiency of propionyl-CoA carboxylase, a heteropolymeric mitochondrial enzyme involved in the catabolism of branched chain amino acids, odd-numbered chain length fatty acids, cholesterol, and other metabolites. The enzyme is composed of alpha and beta subunits which are encoded by the PCCA and PCCB genes, respectively. Mutations in both genes can cause propionic acidemia. The identification of the responsible gene, previous to mutation analysis, can be performed by complementation assay or, in some instances, can be deduced from peculiarities relevant to either gene, including obtaining normal enzyme activity in the parents of many patients with PCCB mutations, observing combined absence of alpha and beta subunits by Western blot of many PCCA patients, as well as conventional mRNA-minus result of Northern blots for either gene or beta subunit deficiency in PCCB patients. Mutations in both the PCCA and PCCB genes have been identified by sequencing either RT-PCR products or amplified exonic fragments, the latter specifically for the PCCB gene for which the genomic structure is available. To date, 24 mutations in the PCCA gene and 29 in the PCCB gene have been reported, most of them single base substitutions causing amino acid replacements and a variety of splicing defects. A greater heterogeneity is observed in the PCCA gene-no mutation is predominant in the populations studied-while for the PCCB gene, a limited number of mutations is responsible for the majority of the alleles characterized in both Caucasian and Oriental populations. These two populations show a different spectrum of mutations, only sharing some involving CpG dinucleotides, probably as recurrent mutational events. Future analysis of the mutations identified, of their functional effect and their clinical relevance, will reveal potential genotype-phenotype correlations for this clinically heterogeneous disorder.

Alternative Splicing↗
PubMed1994

The modification of acetate and propionate side chains during the biosynthesis of haem and chlorophylls: mechanistic and stereochemical studies.

In the conversion of uroporphyrinogen III into protoporphyrin IX and thence into chlorophylls, all eight carboxylic side chains, as well as the four meso positions, are modified, and four enzymes are involved. In the uroporphyrinogen decarboxylase-catalysed reaction all four acetate side chains are converted into methyl groups by the same mechanism, to produce coproporphyrinogen III. Both methylene hydrogen atoms remain undisturbed and the reaction occurs with the retention of stereochemistry. Several questions regarding the enzymology of the decarboxylase are posed. Do all the decarboxylations occur at the same active site and, if so, are the four acetate chains handled in a particular sequence? Is the decarboxylation reaction aided by the transient formation of an electron-withdrawing functionality in the pyrrole ring? Coproporphyrinogen oxidase converts the two propionate side chains of rings A and B into vinyl groups, with an overall anti-periplanar removal of the carboxyl group and the Hsi from the neighbouring position. Evidence is examined to evaluate whether a hydroxylated compound acts as an intermediate in the oxidative decarboxylation reaction. Protoporphyrinogen oxidase then converts the methylene-interrupted macrocycle of protoporphyrinogen IX into a conjugated system. The conversion has been suggested to involve three consecutive dehydrogenation reactions followed by an isomerization step. The face of the macrocycle from which the three meso hydrogen atoms are removed in the dehydrogenation reaction is thought to be opposite to that from which the fourth meso hydrogen is lost during the prototropic rearrangement. In an investigation of the in vivo mechanism for the esterification of the ring D propionic acid group with a C20 isoprenyl group 5-aminolaevulinic acid was labelled with 13C and 18O at C-1 and incorporated into bacteriochlorophyll a. The 18O-induced shift of the 13C resonance in the NMR spectrum showed that both oxygen atoms of the carboxyl group are retained in the ester bond. This and other results suggest that the reaction occurs by the nucleophilic attack of the ring D carboxylate anion on the activated form of an isoprenyl alcohol.

Acetates↗
PubMed2006

Methylmalonic and propionic aciduria.

Methylmalonic and propionic aciduria (PA) are the most frequent forms of branched-chain organic acidurias. These autosomal recessive disorders result from deficient activity of methylmalonyl-CoA mutase and propionyl-CoA carboxylase, respectively. Clinically, acute or chronic neurologic signs are caused by the accumulation of toxic compounds proximal to the metabolic block. Phenotype varies from severe neonatal-onset forms with high mortality and poor outcome to milder forms with a later onset. In both cases the clinical course is dominated by the risk of relapses of life-threatening episodes of metabolic decompensation and of severe organ failure. Despite improvement of treatment, the overall outcome remains disappointing with no major differences between the two diseases. The diagnosis is based on the presence of characteristic compounds in body fluids as detected by organic acid analysis in urine and acylcarnitine profile in blood. Therapy is based on low-protein high-energy diet, carnitine supplementation, and metronidazole. Some patients with methylmalonic aciduria (MMA) respond to pharmacological doses of vitamin B12. Given the poor long-term prognosis, liver transplantation has been recently attempted as an alternative therapy to conventional medical treatment to cure the underlying metabolic defect. Nevertheless, the overall experience to date does not clearly demonstrate its effectiveness in preventing further deterioration or improving survival and quality of life. The recent implementation of neonatal screening by electrospray tandem mass spectrometry has decreased early mortality and improved the short-term outcome, without changing the detection rate of both diseases in the screening population compared to clinically detected cases. However, the limited number of patients and the short duration of their follow-up do not yet permit drawing final conclusions on its effect on the long-term outcome of methylmalonic and propionic acidemia.

Humans↗
PubMed2005

Effects of acetate and propionate on the performance of a photosynthetic biofilm reactor for sulfide removal.

The effects of acetate and propionate on the performance of a recently proposed and characterized photosynthetic biological sulfide removal system have been investigated with a view to predicting this concept's suitability for removing sulfide from wastewater undergoing or having undergone anaerobic treatment. The concept relies on substratum-irradiated biofilms dominated by green sulfur bacteria (GSB), which are supplied with radiant energy in the band 720-780 nm. A model reactor was fed for 7 months with a synthetic wastewater free of volatile fatty acids (VFAs), after which time intermittent dosing of the wastewater with acetate or propionate was begun. Such dosing suppressed the areal net sulfide removal rate by approximately 50%, and caused the principal net product of sulfide removal to switch from sulfate to elemental-S. Similarly suppressed values of this rate were observed when the wastewater was dosed continuously with acetate, and this rate was not significantly affected by changes in the concentration of ammonia-N in the feed. The main net product of sulfide removal was again elemental-S, which was scarcely released into the liquid, however. Sulfate reduction and sulfur reduction were observed when the light supply was interrupted and were inferred to be occurring within the irradiated biofilm. A preexisting conceptual model of the biofilm was augmented with both of these reductive processes, and this augmented model was shown to account for most of the observed effects of VFA dosing. The implications of these findings for the practicality of the technology are considered.

Acetates↗
PubMed2006

Propionate-regulated high-yield protein production in Escherichia coli.

A new expression system containing the Salmonella enterica prpBCDE promoter (P(prpB)) responsible for expression of the propionate catabolic genes (prp BCDE) and prpR encoding the positive regulator of this promoter has been developed and tested. The main features of the expression system compared to those based on the bacteriophage T7 promoter are low background expression and high induced expression in Escherichia coli strains BL21, BL21(DE3), MG1655, and W3110. In addition, propionate is an inexpensive, simple-to-use, nontoxic inducer that is attractive for large-scale protein production. Hence, this new system is highly complementary to the widely used T7 promoter-driven expression systems.

Bacteriophage T7↗
PubMed1979

The identification of 3-keto-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid in a patient with propionic acidemia.

Two abnormal metabolites, 3-keto-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid, have been identified and quantitated in the urine of a child with propionic acidemia. These metabolites may be produced as a result of the self-condensation of propionyl-CoA. Data are presented to show that the unusual ketone, 3-pentanone, which has been observed previously in the urine of patients with propionic acidemia, is produced as a result of the decarboxylation of 3-keto-2-methylvaleric acid.

Gas Chromatography-Mass Spectrometry↗
PubMed1983

The occurrence of substituted 3-methyl-3-hydroxyglutaric acids in urine in propionic acidaemia and in beta-ketothiolase deficiency.

THe urine of two patients with propionic acidaemia contained 3-ethyl-3-hydroxyglutaric acid which has a propionyl residue in place of one of the acetyl residues of the normal metabolite 3-methyl-3-hydroxyglutaric acid. Related compounds, 2,3-dimethyl-3-hydroxyglutaric acid, 2-methyl-3-ethyl-3-hydroxyglutaric acid and 2,3,4-trimethyl-3-hydroxyglutaric acid, could not be detected in propionic acidaemia urine, but 2,3-dimethyl-3-hydroxyglutaric acid was excreted by a patient with beta-ketothiolase deficiency.

Acetyl-CoA C-Acyltransferase↗
PubMed1992

Acute effects of topical methyl tert-butyl ether or ethyl propionate on gallbladder histology in animals: a comparison of two solvents for contact dissolution of cholesterol gallstones.

Experiments were performed in anesthetized rabbits and piglets to assess gallbladder mucosal injury during irrigation with methyl tert-butyl ether, a C5 ether, or ethyl propionate, a C5 ester--two organic solvents used in the contact dissolution of cholesterol gallstones. In 44 New Zealand White rabbits, the gallbladder was exposed to individual solvents or saline solution through a transhepatic catheter for 2 hr. Gallbladders were then harvested and fixed immediately or after a recovery period of 1, 4 or 8 days. Tissue sections were examined under light microscopy, and severity of injury was graded with predefined criteria by two pathologists blinded to the animals' treatment regimens. Histological assessment showed severe mucosal injury such as necrosis of the cells at the villus tips immediately after 2 hr of exposure to either solvent. After 4 days, injury had decreased significantly; after 8 days, complete mucosal healing had taken place. A similar study was performed in 32 piglets. Solvent or saline solution was oscillated in and out of the gallbladders of these piglets with a computer-controlled syringe pump at a pressure less than the leakage pressure of the gallbladder. Histological assessment was performed on tissue samples obtained immediately after the procedure or 8 days later. Both solvents caused severe mucosal injury; however, after 8 days complete mucosal healing had occurred, so that gallbladders exposed to solvent were indistinguishable from gallbladders exposed to saline solution, which was used as control. We conclude that both methyl tert-butyl ether and ethyl propionate cause moderate to severe epithelial injury but that the gallbladder epithelium regenerates within a few days.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed2002

Propionic acidemia: analysis of mutant propionyl-CoA carboxylase enzymes expressed in Escherichia coli.

Deficiency of propionyl-CoA carboxylase (PCC) results in propionic acidemia, an autosomal recessive disorder characterized by ketoacidosis sufficiently severe to cause neonatal death. PCC is involved in the catabolism of branched-chain amino acids, odd-chain fatty acids, and cholesterol. The enzyme is a biotin-dependent mitochondrial protein composed of two heterologous subunits arranged into an 800-kDa alpha(6 )beta(6) dodecameric structure. Approximately 60 mutations have been reported in the nuclear genes PCCA and PCCB that encode the two PCC subunits. The vast majority of these mutations have not been examined at the protein level. We present an initial characterization of 13 mutations located in exons 1, 3-7, and 12-14 of PCCB. After expression in E. coli, these recombinant mutant enzymes were analyzed for stability, biotinylation, alpha-beta subunit interaction, and activity. Our results show a functional dichotomy in these PCCB mutations with some mutants (R44P, S106R, G131R, G198D, V205D, I408del, and M442T) capable of varying degrees of assembly but forming catalytically inactive PCC proteins. Other PCCB mutants (R165W, E168K, D178H, P228L, and R410W) that are PCC deficient in patient-derived fibroblasts, were found to be capable of expressing wild-type level PCC activity when assembled in our chaperone-assisted E. coli expression system. This result indicates that these mutations exert their pathogenic effect due to an inability to assemble correctly in patients' cells. This initial screen has identified a range of mutant PCC proteins that are sufficiently stable to be purified and subsequently used for structure-function analysis to further elucidate the complex relationship between genotype and phenotype in propionic acidemia.

Amino Acid Metabolism, Inborn Errors↗
PubMed1995

Apoptosis in colorectal tumour cells: induction by the short chain fatty acids butyrate, propionate and acetate and by the bile salt deoxycholate.

The short chain fatty acids acetate, propionate and butyrate are produced when dietary fibre is fermented by the colonic bacteria. We have previously shown that sodium butyrate induces apoptosis in 3 colorectal tumour cell lines. We have extended our study to 3 adenoma and 4 carcinoma cell lines and investigated whether propionate and acetate also induce apoptosis. All 3 short chain fatty acids induced apoptosis at physiological concentrations, but of the 3, butyrate was the most effective. Since these fatty acids are produced as a result of bacterial fermentation of dietary fibre, this may in part explain the correlation between a high-fibre diet and low colorectal cancer incidence. Sodium butyrate induced apoptosis in all 7 of the cell lines studied; however, 2 of the 4 carcinoma cell lines (PC/JW/FI and S/KS/FI) were more resistant to butyrate-induced apoptosis than the 3 adenoma cell lines, suggesting that at least some carcinomas may evolve mechanisms to protect the cells from the induction of apoptosis. The bile acid deoxycholic acid has previously been reported as a possible tumour promoter in the large intestine and its levels are reduced by dietary fibre. Concentrations of between 10 nM and 0.1 mM had no effect on either the proliferation or apoptosis of colonic tumour cells in vitro. However, a significant induction of apoptosis was obtained at a concentration of 0.5 mM. These results may have significance for the aetiology of colorectal cancer.

Acetates↗
PubMed2001

Acetate, propionate and butyrate in plasma: determination of the concentration and isotopic enrichment by gas chromatography/mass spectrometry with positive chemical ionization.

This study describes a rapid and simple method to determine short-chain fatty acid (SCFA) concentrations and their isotopic enrichments (M(0) + 1 and M(0) + 2) in human plasma. Sample preparation involves SCFA extraction and derivatization with 1-(tert-butyldimethylsilyl)imidazole. Gas chromatography/mass spectrometry was performed using chemical ionization with ammonia as the reagent gas. Outstanding resolution, excellent linearity and good detection limits were obtained. Inter-assay and intra-assay repeatability was below 10% and 3% respectively for SCFA concentration. Inter-assay repeatability was below 5%, 4%, 6%, and 14% for isotopic enrichment determination of [1-(13)C]acetate and [1,2-(13)C(2)]acetate, [1-(13)C]propionate and [1-(13)C]butyrate respectively, with intra-assay being below 6%. Such SCFA concentrations and isotopic enrichments were determined in the plasma of rats infused with a (13)C-labeled SCFA. The turnovers of acetate, propionate and butyrate in rats were 19 micromol kg(-1) min(-1), 2.6 micromol kg(-1) min(-1), 0.3 micromol kg(-1) min(-1) respectively.

Acetates↗
PubMed1988

Prenatal diagnosis of propionic acidemia in chorionic villi by direct assay of propionyl CoA carboxylase.

Prenatal diagnosis of propionic acidemia was achieved by the direct assay of propionyl CoA carboxylase in chorionic villi. The diagnosis was confirmed by determination of methylcitrate in amniotic fluid and measurement of propionyl CoA carboxylase in the liver from the abortion. Discrepancy between [14C]-propionate incorporation into protein of chorionic villi or cultured chorionic cells and propionyl CoA carboxylase activity is reported.

Amino Acid Metabolism, Inborn Errors↗
PubMed2006

Sorption of chlorophenoxy propionic acids by organoclay complexes.

The organoclays Wyoming montmorillonite (SWy-1, Mon), W. R. Grace vermiculite (Ver), and Tung-Wei (Tw) soil montmorillonite were prepared to sorb or remove chlorophenoxy propionic acids (CPA) pollutants such as 2,4-dichlorophenoxy acid (2,4-DP), 2,4,6-tri chlorophenoxy acid (2,4,6-TCP), and pentachlorophenoxy propionic acid (PCP). The objective of this study was to evaluate the behavior of organoclays that have sorbed three types of CPA and to assess the applicability to basaltic-developed iron-rich calcareous soil. This type of soil contains high amounts of montmorillonite. The mean-layer charge per formula unit of Mon, Ver, and Tw clays, characterized by the alkylammonium method, was 0.27, 0.70, and 0.52 mol(c)/O(10)(OH)(2), respectively. The sorption isotherms of 2,4-DP, 2,4,6-TCP, and PCP sorbed by hexadecyltrimethyl-ammonium (HDTMA organoclay, 150% cation-exchange capacity) were evaluated. The data on sorption of CPAs fit well with the Freundlich isotherm equation. In general, the sorption of three types of CPA by the organoclays showed a linear relationship, with a high correlation coefficient (r > 0.935). The K(f) value of PCP was the same as that of 2,4,6-TCP sorbed by the organoclays and was higher than that of 2,4-DP. However, the 1/n(f) values of the three CPAs sorbed by the organoclays did not show significant differences. In general, maximum CPA sorption occurred at a pH of approximately 3, but very significant differences were observed for the 2,4,6-TCP and PCP sorbed by the organoclays. However, there was no significant difference in pH for 2,4-DP sorption. This should be related to the solubility and pKa of each CPA. The experimental results showed that solubility of 2,4-DP, 2,4,6-TCP, and PCP is a function of pH, ionic strength, and temperature.

Adsorption↗
PubMed1994

Correction of the metabolic defect in propionic acidemia fibroblasts by microinjection of a full-length cDNA or RNA transcript encoding the propionyl-CoA carboxylase beta subunit.

Propionyl-CoA carboxylase (PCC) is a mitochondrial, biotin-dependent enzyme, composed of an equal number of alpha and beta subunits, that functions in the catabolism of branched-chain amino acids and other metabolites. Mutations of the PCCA (alpha subunit) or PCCB (beta subunit) gene cause the inherited metabolic disease, propionic acidemia. We report the cloning of a full-length cDNA encoding the beta subunit of human PCC. The open reading frame encodes a pre-beta polypeptide of 539 amino acids (58,205 Da). The cDNA was introduced into the expression vector, pRc/CMV, and microinjected into the nucleus or, as ribotranscripts, into the cytoplasm of fibroblast lines from patients with defects of the beta subunit. The restoration of function was monitored by autoradiography of PCC-dependent [14C]-propionate incorporation into cellular protein. These results confirm the completeness of the clone and demonstrate the capacity for beta subunits derived from the microinjected cDNA or RNA to be transported into mitochondria and assembled with endogenously derived alpha subunits to form functional PCC.

Amino Acid Sequence↗