Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “GLUTARATES”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,171 records · Page 65Linked to original sources
PubMed1998

[3-Hydroxy-3-methyl-glutaryl-CoA-lyase deficiency as coma etiology in the neonatal period: case report].

We report a patient that presented two episodes of coma in the neonatal period, with severe metabolic acidosis and hypoglycemia, without ketosis. The urinary organic acid analysis showed increased amounts of 3-hydroxy-3-methyl-glutaric, 3-methylglutaconic, 3-methylglutaric and 3-hydroxyisovaleric acid. The deficiency of 3-hydroxy-3-methylglutaryl-CoA lyase was diagnosed by the clinical and metabolic features. This disease shows autosomal recessive inheritance and the treatment is done by a diet with restriction of protein (mainly leucine) and lipids, high in carbohydrate content, and the avoidance of fasting and carnitine supplementation.

Coma↗
PubMed2001

Inhibition of in vitro CO2 production and lipid synthesis by 2-hydroxybutyric acid in rat brain.

2-Hydroxybutyric acid appears at high concentrations in situations related to deficient energy metabolism (e.g., birth asphyxia) and also in inherited metabolic diseases affecting the central nervous system during neonatal development, such as "cerebral" lactic acidosis, glutaric aciduria type II, dihydrolipoyl dehydrogenase (E3) deficiency, and propionic acidemia. The present study was carried out to determine the effect of 2-hydroxybutyric acid at various concentrations (1-10 mM) on CO2 production and lipid synthesis from labeled substrates in cerebral cortex of 30-day-old Wistar rats in vitro. CO2 production was significantly inhibited (30-70%) by 2-hydroxybutyric acid in cerebral cortex prisms, in total homogenates and in the mitochondrial fraction. We also demonstrated a significant inhibition of lipid synthesis (20-45%) in cerebral cortex prisms and total homogenates in the presence of 2-hydroxybutyric acid. However, no inhibition of lipid synthesis occurred in homogenates free of nuclei and mitochondria. The results indicate an impairment of mitochondrial energy metabolism caused by 2-hydroxybutyric acid, a fact that may secondarily lead to reduction of lipid synthesis. It is possible that these findings may be associated with the neuropathophysiology of the situations where 2-hydroxybutyric acid is accumulated.

Analysis of Variance↗
PubMed1992

Specificity of basolateral organic anion exchanger in proximal tubule for cellular and extracellular solutes.

We used an epifluorimetric technique to study the mechanism of organic anion transport across the basolateral surface of isolated S2 segments of rabbit proximal tubules. Fluorescein influx and efflux rates across the basolateral surface of lumen-collapsed tubules were determined from serial measurements of cellular fluorescein content after its addition to or removal from the bathing medium. We examined the effect on fluorescein transport of monocarboxylic and dicarboxylic metabolic intermediates added to the bathing medium or preloaded into the cells. The presence of the monocarboxylates (octanoate, valerate, butyrate, propionate, and acetate) in the bathing medium inhibited fluorescein influx. The eight-carbon-chain fatty acid, octanoate, was nearly as potent an inhibitor as probenecid and was more potent than p-aminohippurate (PAH) (IC50 = 10, 6, and 141 microM, respectively); the shorter chain fatty acids were much less effective (IC50 greater than or equal to 1,000 microM). The dicarboxylates (succinate, adipate, and alpha-ketoglutarate) moderately inhibited fluorescein influx (IC50 = 505, 245, and 257 microM, respectively). To determine the effect of intracellular organic anions on fluorescein influx, tubules were preincubated with organic anions to load the cells; the compounds were then removed from the bath, and fluorescein influx was measured. Preincubation with monocarboxylates, octanoate, butyrate, acetate, and PAH had no effect. In contrast, the dicarboxylates alpha-ketoglutarate, glutarate, and adipate stimulated fluorescein influx by 58, 53, and 43%, respectively, but succinate had no effect. Fluorescein efflux was accelerated by medium octanoate, PAH, alpha-ketoglutarate, and succinate but not by acetate. Probenecid alone had no effect on fluorescein efflux, but it blocked the stimulation caused by medium PAH.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMedDate not supplied

Verrucas. Guidelines for management.

Most people will experience infection with human papilloma virus (HPV) at some point in their life. Diagnosis, based on clinical examination, is usually straight forward. Treatment can, however, be challenging. Indications for treatment include pain, interference with function, cosmetic embarrassment, and risk of malignancy. Clearance rates are highest in young, healthy individuals with short duration of infection. Treatment may be with destructive agents (keratolytics, cryotherapy, curettage and cautery, laser, photodynamic therapy), with antimitotic agents (podophyllin, bleomycin, retinoids), with immune stimulants (topical sensitizers, cimetidine), or with topical virucidal agents [formaldehyde (formalin), glutaral (gluteraldehyde)]. As yet, there is no single totally effective treatment for viral warts. Some patients may choose to leave their warts untreated until spontaneous resolution. In those who seek intervention, simple, well tolerated therapies should be chosen initially in preference to more complicated, potentially harmful agents. It is likely that future research will be directed to developing an antiviral agent specific for HPV which would be safe, effective and not prohibitively expensive.

Clinical Protocols↗
PubMed1980

Red cell sorbitol: an indicator of diabetic control.

Intact human erythrocytes accumulate intracellular sorbitol in response to the medium's glucose concentration during in vitro incubations. Sorbitol was identified and measured both enzymatically and by gas-liquid chromatography. The sorbitol produced is most likely a result of the activity of aldose reductase, since (1) a low glucose concentration in the medium elicits this response, and (2) this activity is completely blocked by tetramethylene glutaric acid, a specific inhibitor of aldose reductase. Erythrocyte sorbitol levels in insulin-dependent diabetics are clearly above those of nondiabetics after an 8 h fast. A good correlation exists between red cell sorbitol content and coincident plasma glucose concentrations. Individual exceptions to this rule exist, however, and suggest that red cell sorbitol levels may provide information about in vivo polyol pathway activity that may be important in the pathogenesis of diabetes-associated complications.

Blood Glucose↗
PubMedDate not supplied

Ultrastructure of synapses in the lateral line canal organ.

The ultrastructure of afferent and efferent synapses on hair cells in the lateral line canal organ of hair cells in the lateral line canal organ of the fish Lota lota was studied, utilizing various fixation and staining techniques. New information was obtained about membrane-associated material, such as the presynaptic body and postsynaptic densities, by examining glutaral-dehyde-fixed material not subjected to osmication. Contrast was instead obtained either by section staining with uranyl acetate and lead citrate or by block impregnation with phosphotungstic acid (PTA). PTA-staining enhances the postsynaptic membrane of both the afferent and efferent synapses. It also stains the presynaptic dense projections at the efferent synapse and in a differential fashion, the afferent synaptic body. Secretion staining reveals a substructure in the feed of the afferent synaptic body, faintly seen but masked in osmicated tissue.

Animals↗
PubMed1981

Propionyl-CoA carboxylase deficiency: case report, effect of low-protein diet and identification of 3-oxo-2-methylvaleric acid 3-hydroxy-2-methylvaleric acid, and maleic acid in urine.

Vomiting, lethargy and metabolic acidosis were the main initial symptoms of metabolic disease in a 1 month old girl. Her older sister had died from a similar disease, considered to be Reye's syndrome, at an age of 15 months. The urine of the present case contained 2-methylcitric acid, 3-hydroxypropionic acid, N-propionylglycine, 2-hydroxy-3-methylbutyric acid, N-tiglylglycine, 3-hydroxyvaleric acid and glutaric acid. These metabolites are all known to be associated with propionyl-CoA accumulation. Free propionic acid was not detected in the urine. In addition, the urine contained 3-oxo-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid, probably formed by condensation of two molecules of propionyl-CoA. The identity of these metabolites was confirmed by synthesis. An elevated urinary concentration of maleic acid and fumaric acid was another constant abnormality. The activity of propionyl-CoA carboxylase in leucocytes was about 20% of the normal activity. The girl was teated with a low-protein diet since the diagnosis was made at an age of 1 month, and her psychomotor development was satisfactory at an age of 2 1/2 years. She had a few episodes of acidosis during infections.

Amino Acid Metabolism, Inborn Errors↗
PubMed1976

Metabolism of linoleamides. I. Absorption, excretion and metabolism of N-(alpha-methylbenzyl)linoleamide in rat and man.

1. In urine of rats dosed with N-(alpha-methylbenzyl)linoleamide (MBLA), three dicarboxylic acid monoamides, N-(alpha-methylbenzyl)succinic acid monoamide, N-(alpha-methylbenzyl)glutaric acid monoamide and N-(alpha-methylbenzyl)adipic acid monoamide, were identified. Conjugated alpha-methylbenzylamine, hippuric acid and conjugates of the dicarboxylic acid monoamides were also found in the urine. N-(alpha-Methylbenzyl)succinic acid monoamide was the main metabolite in rats. 2. Biliary excretion of radioactivity was studied in rats, cannulated for collection of bile and duodenal infusion, after oral administration of N-(alpha-methylbenzyl)[1-14C]linoleamide. With constant duodenal infusion of bile, about 7% of the dose was excreted in the bile, while excretion of radioactivity was negligible without bile infusion. 3. The g.l.c. analysis of human urine after oral administration of MBLA revealed that two dicarboxylic acid monoamides were present and N-(alpha-methylbenzyl)succinic acid monoamide was the main metabolite. 4. MBLA was excreted unchanged in the faeces of men who received MBLA to the extent of about 53% dose in 3 days. 5. MBLA was not detected (less than 1 mug/ml) in the serum of a volunteer who had been taking an oral daily dose of 1500 mg of MBLA for 3 months.

Administration, Oral↗
PubMed1976

Metabolism of linoleamides. II. Absorption, distribution, excretion and metabolism of N-[alpha-phenyl-beta-(p-tolyl)ethyl]linoleamide.

1. Absorption, distribution, excretion and metabolism of (-)N-[alpha-phenyl-beta-(p-tolyl)ethyl][14C]linoleamide (14C-PTLA) were studied in rats and dogs. Faecal excretion of PTLA was studied in dogs and men by g.l.c. 2. 14C-PTLA (10 mg/kg) given orally to rats resulted in urinary and faecal excretion of radioactivity of 2 and 93%, respectively, by male rats and 8 and 87% by female rats in 48 h. Faecal excretion of PTLA in men was similar to that in rats. 3. Distribution of radioactivity in rats and dogs after oral administration of 14C-PTLA showed that a major part of the dose was not absorbed. 4. N-[alpha-phenyl-beta-(p-tolyl)ethyl]glutaric acid monoamide were detected in the urine of rats dosed orally with 14C-PTLA.

Administration, Oral↗
PubMed1992

Stabilities and properties of multilinked hemoglobins.

Crosslinking of both human and dog hemoglobin has been done with a variety of reagents to produce singly, doubly and multiply crosslinked hemoglobins. Succinate and glutarate diaspirins did not crosslink deoxy human hemoglobin in good yield, in contrast to the fumarate analog (DBSF). Deoxy dog Hb did not react well with DBSF, but oxy dog Hb did react, giving crosslinked tetramers as well as dimers on SDS electrophoresis. Crosslinking with a short, rigid reagent (difluorodinitrobenzene) resulted in a similar product for both oxy and deoxy hemoglobin that had high stability and oxygen affinity. The trilinker, tris-chloroethylamine, produced a more stable product than the corresponding crosslinker, bis-chloroethylamine. Double crosslinking oxy Hb with DBSF and dimethylpimelimidate or with DBSF followed by deoxygenation and recrosslinking with DBSF gave products with higher denaturation temperatures. The diaspirin double crosslinked product had high oxygen affinity.

Animals↗
PubMed1997

Arginase in lactating bovine mammary glands: implications in proline synthesis.

The occurrence and subcellular distribution of arginase have been studied in mammary glands from lactating dairy cattle. The enzyme appears to be localized in the mitochondrial fraction, although a significant amount has been found to be associated with the cytosolic fraction. Both mitochondrial and cytosolic arginase are activated by heating with Mn2+. The Michaelis constants for the two fractions, however, are different: 49.5 and 18.5 mM for the mitochondrial fraction and cytosolic fraction, respectively. Overall the total enzyme concentration in the gland suggests that these enzymes contribute to the conversion of arginine to ornithine. Ornithine, in turn, may be converted by ornithine aminotransferase into an intermediate, L-delta 1-pyrroline-5-carboxylate; concurrently. alpha-keto-glutarate is transformed into glutamic acid. Finally, pyrroline-5-carboxylate reductase yields proline, an important amino acid that is needed for casein synthesis. Because pyrroline-5-carboxylate reductase requires NADPH, and because ornithine aminotransferase uses alpha-ketoglutarate, this new pathway is linked to the Krebs cycle through the cytosolic isocitrate dehydrogenase, which is the source of both of these intermediates.

Animals↗
PubMed1992

Sodium-sensitive, probenecid-insensitive p-aminohippuric acid uptake in cultured renal proximal tubule cells of the rabbit.

In the intact kidney, renal proximal tubule cells accumulate p-aminohippurate (PAH) via a basolateral, probenecid- and sodium-sensitive transport system. Primary cultures of rabbit proximal tubule cells retain sodium-glucose co-transport in culture, but little is known about PAH transport in this system. Purified proximal tubule cells from a rabbit were grown in culture and assessed for PAH and alpha-methyl-D-glucoside uptake capacities as well as proximal tubule marker enzyme activities. Control PAH uptake on collagen-coated filters (20 +/- 3 pmol/mg protein.min; n = 8) was not significantly different from uptake in the presence of 1 mM probenecid (19 +/- 4 pmol/mg protein.min; n = 8). Uptake from the basal side of the cell was 3.9 +/- 0.7 times greater than that from the apical side. In multi-well plate studies, the uptake was significantly reduced by removing sodium from the medium and stimulated by coating the wells with collagen. Glutarate (10 mM) had no effect on the uptake of PAH. Other differentiated proximal tubule characteristics were retained in culture, including the ability to form domes and to transport glucose by a phlorizin-sensitive system. Phlorizin-sensitive 1 mM alpha-methyl-D-glucoside uptake was 134 +/- 42 pmol/mg protein.min (n = 7; P less than 0.02). The proximal tubule marker enzymes alkaline phosphatase and gamma-glutamyltranspeptidase, increased in activity in the cultures after confluence. It was concluded that whereas some differentiated properties were retained during primary culture of rabbit proximal tubule cells, the PAH transport system was selectively lost or modified from that present in the intact kidney.

Alkaline Phosphatase↗
PubMed2003

Construction and expression of a humanized M2 autoantigen trimer and its application in the diagnosis of primary biliary cirrhosis.

AIM: To construct and express a humanized M(2) autoantigen trimer designated as BPO and to apply it in the diagnosis of primary biliary cirrhosis (PBC). METHODS: cDNA fragments encoding M(2)-reactive epitopes of pyruvate dehydrogenase complex E(2) (PDC-E(2)), branched chain 2-oxo-acid dehydrogenase complex E(2) (BCOADC-E(2)) and 2-oxo-glutarate dehydrogenase complex E(2) (OGDC-E(2)) were amplified with PCR using total RNA extracted from human peripheral mononuclear blood cells. The fragments were cloned into the plasmid vector pQE-30 and then transferred into E. coli M15 (pREP4) for expression, which was induced by isopropylthio-beta-D-galactoside. The expressed recombinant BPO protein was demonstrated by SDS-PAGE, Western-blotting and Immunoabsorption test, its antigenic reactivity and specificity were identified with seven M(2)-positive sera confirmed at Euroimmun Research Center (Germany). Using the purified BPO, M(2) antibodies in sera from patients with PBC and other liver related diseases were detected with ELISA. RESULTS: The expressed BPO was observed with both antigenic reactivity and specificity of M(2) autoantigens. The determination of M(2) antibodies by BPO with ELISA was more sensitive than using the Euroimmun's kit with the coefficients of variation less than 10 % in both interassay and intraassay. With the newly established method, M(2) antibodies were found in 100 % (20/20) of patients with PBC. Six cases of liver disease with unknown etiology and 1 patient with drug induced liver injury had detectable levels of serum M(2) antibodies. There were also 2 patients with autoimmune cholangitis and 1 with autoimmune hepatitis showing M(2)-antibody positive. CONCLUSION: Compared with the routine immunofluorescence assay and commercially available assay kit using porcine heart mitochondrial protein as the antigen, the detection system established in the present study shows higher sensitivity and specificity and may be used as a powerful tool for the diagnosis of PBC.

Autoantibodies↗
PubMed1982

Characterization of cerebral cysteine sulfinic acid decarboxylase. Molecular parameters and inhibition studies.

The molecular properties of cysteine sulfinic acid decarboxylase (EC 4.1.1.29) from calf brain were examined in a crude enzyme preparation. The Stokes radius (42 A) was determined by gel filtration and the sedimentation coefficient, s20,w (6.1 x 10(-13 s) by density gradient centrifugation. Calculation of the molecular weight and frictional ratio gave values of 73,000 and 1.52, respectively. beta-Mercaptopropionic acid and an arginine reagent, phenylglyoxal, were observed to be potent inhibitors of the enzyme. Pyridoxal phosphate, a cofactor of the decarboxylase, was observed to protect the enzyme against phenylglyoxal inhibition. This result indicates that an arginine residue may be located at the active site of cysteine sulfinic acid decarboxylase. The components of taurine metabolism gave little or no inhibition of the decarboxylase. Glutaric acid, malic acid and C-allylglycine, all widely known as potent inhibitors of glutamic acid decarboxylase, only slightly inhibited cysteine sulfinic acid decarboxylase.

3-Mercaptopropionic Acid↗
PubMed1976

Aspects of mitochondrial activity in the estuarine bivalves Crassostrea rhizophorae and Lucina pectinatus: a comparative approach.

In identical laboratory conditions, some differences in the endogenous relative activity (mul O2/mg protein/ml/h) were determined by the addition of succinate and alpha-keto-glutarate to the mitochondrial-rich-fraction from heart and both parts of the adductor muscle in oyster (Crassostrea rhizophorae) and from heart and adductor muscle in clams (Lucina pectinatus).

Animals↗
PubMed1999

Transport properties of nonsteroidal anti-inflammatory drugs by organic anion transporter 1 expressed in Xenopus laevis oocytes.

Organic anion transporter 1 (OAT1) is the para-aminohippurate (PAH) transporter in the basolateral membrane of the proximal tubule. The present study investigated whether or not nonsteroidal anti-inflammatory drugs (NSAIDs) are transported by OAT1. All of the NSAIDs tested inhibited [14C]PAH uptake via OAT1 expressed in Xenopus laevis oocytes. Ibuprofen, indomethacin, salicylurate, and naproxen showed the strongest potency to inhibit [14C]PAH uptake (Ki approximately 2-10 microM); acetylsalicylate, salicylate, and phenacetin exhibited moderate potency (Ki approximately 300-400 microM), and acetaminophen (paracetamol) exhibited the weakest inhibitory potency (Ki approximately 2 mM). Radiolabeled acetylsalicylate, salicylate, and indomethacin were taken up by OAT1 and the uptake rate of these three NSAIDs was enhanced by the outwardly directed dicarboxylate gradient. The efflux of the preloaded [14C]PAH from the oocytes via OAT1 was trans-stimulated by PAH and glutarate added to the media. The addition of salicylate, acetylsalicylate, or salicylurate into the media also trans-stimulated the efflux of PAH, whereas indomethacin did not. The present study indicates that OAT1 is responsible for the renal uptake and secretion of NSAIDs.

Animals↗
PubMedDate not supplied

[Immobolization of beta-galactosidase on silochrome].

Beta-Galactosidase (beta-D-galactoside galactohydrolase 3.2.1.23) from Curvularia inaequalis was immobilized by glutaric dialdehyde on gamma-aminopropyl triethoxysilane treated porous siliceous carrier silochrome. From the crude preparation with a specific activity of 3.1 U/mg immobilized beta-galactosidase with an activity of 113 U/g was obtained. The immobilized enzyme did not show significant changes in its enzymic properties. The column filled with the resultant preparation and used to hydrolyze lactose in milk whey maintained 50% of its initial activity after a 30-day work at 50 degrees C.

Catalysis↗