Structure, properties, spectra, suppliers and links for: Glutaric acid, 110-94-1. Baric I, Wagner L, Feyh P, Liesert M, Buckel W, Hoffmann GF. 25,26 It is used in the production of commercial polyesters and polyamides. In aqueous solution, the equilibrium of acid dissociation can be written symbolically as: HA + H 2 O = A-+H 3 O + . GA1 is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH). Patients have macrocephaly and can develop striatal injury usually between 6 months and 6 years which is usually related to illness or catabolic stress and can lead to a movement disorder. Metabolic engineering of Corynebacterium glutamicum for the production of glutaric acid, a C5 dicarboxylic acid platform chemical. Metab Eng, 51:99-109, 23 Aug 2018 Business listings of Glutaric Acid, 110-94-1 manufacturers, suppliers and exporters in Mumbai, ग्लूटेरिक एसिड विक्रेता, मुंबई, Maharashtra along with their contact details & address. Table of Acids with Ka and pKa Values* CLAS Acid HA A-Ka pKa Acid Strength Conjugate Base Strength Hydroiodic HI I-Hydrobromic HBr Br-Perchloric HClO4 ClO4-Hydrochloric HCl Cl-Chloric HClO3 ClO3-Sulfuric (1) H2SO4 HSO4-Nitric HNO3 NO3-Strong acids completely dissociate in aq solution (Ka > … Calculate the pH of a solution containing 0.0347 M glutaric acid and 0.020 M potassium hydrogen glutarate. Here we report metabolic engineering of an l-lysine–overproducing Corynebacterium glutamicum strain for the high-level production of glutaric acid. Glutaric aciduria type I (GA-I) is a rare cerebral organic acid disorder caused by inherited deficiency of glutaryl-CoA dehydrogenase (GCDH; EC 1.3.99.7), a mitochondrial flavoprotein catalysing the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA in the final catabolic pathways of the amino acids l-lysine, l-hydroxylysine and l-tryptophan (Goodman et al., 1975). This problem has been solved! 27 Moreover, it is built into organometallics, leading to antimicrobial agents, 28 transistors, and capacitors. These free radicals have been investigated by electron nuclear double resonance (ENDOR) technique. … Interruption of this leads to an inability to produce Acetyl‐CoA, which is further metabolized before partaking in the Krebs cycle. All data apply to dilute aqueous solutions and are presented in the form of pK a , which is the negative of the logarithm of the acid dissociation constant K a . See the answer. A large Ka value indicates a strong acid because it means the acid is largely dissociated into its ions. A large Ka value also means the formation of products in the reaction is favored. Chronically high levels of glutaconic acid are associated with glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1). The solubility of the cocrystal and pure compounds has been determined in chloroform and acetonitrile. GA1 is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH). Get contact details & address of companies manufacturing and supplying Glutaric Acid, 110-94-1 across India. Glutaric acid, also known as pentanedioic acid, is a carboxylic acid that is widely used for various applications including the production of polyesters, polyamides, polyurethanes, glutaric anhydride, 1,5-pentanediol, and 5-hydroxyvaleric acid. The enzymatic defect results in elevated concentrations of specific organic acids, such as glutaric acid, 3‐hydroxyglutaric acid, glutaconic acid, and specific carnitines conjugates, such as glutaryl carnitine, in body tissues, which can all be reliably detected by biochemical analyses using gas chromatography/mass spectrometry (organic acids) and tandem mass spectrometry (acylcarnitines). The mass fraction of GA (wGA) ranged from (0 to 30.11) %. Complete gene sequencing (amplification by polymerase chain reaction and sequencing using BigDye Terminator v3.1 and capillary electrophoresis) was performed for one of the patients, and this revealed a novel mutation in the gene for GA-1, i.e. On exposure to X-rays, glutaric acid crystals generate two stable free radicals. Glutaric acid is a pentanedioic acid. Chronically high levels of 3-hydroxyglutaric acid are associated with glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1). A limitation of this analytic method is its inability to differentiate between several isomers. Use under medical supervision. An acid dissociation constant, K a, is a quantitative measure of the strength of an acid in solution.It is the equilibrium constant for a chemical reaction known as dissociation of acid–base reactions. Presence of glutaric acid in urine and plasma is an indicator of type I glutaric aciduria (GA-I). Glutaric acid is an important dicarboxylic acid used in the synthesis of commercial polymers. The copolymers of diacid, for example, glutaric acid, hexanedioic acid and decanedioic acid, with diols, for example, 1,3-propylene glycol, 1,4-butanediol and 1,6-hexanediol have heat-absorbing and -releasing properties. As their name suggests, polyprotic acids contain more than one acidic proton. The phenotypic spectrum of untreated glutaric acidemia type 1 (GA-1) ranges from the more common form (infantile-onset disease) to the less common form (later-onset disease – i.e., after age 6 years). Find here Glutaric Acid, 110-94-1 suppliers, manufacturers, wholesalers, traders with Glutaric Acid prices for buying. In the present study, the potential cytotoxic effects of GA (0.1~50 mM for 24~96 h) were examined in cultured primary rat striatal neurons. In order to provide thermodynamic data for mixed dibasic acid separation technology, the solubilities of adipic acid (AA) and succinic acid (SA) in glutaric acid (GA) + acetone mixture and GA + n-butanol mixture were measured by dynamic method at atmospheric pressure. The Ka Values For Glutaric Acid Are 4.52 × 10-5 (Ka1) And 3.78 × 10-6 (Ka2). HGEMP : Acylcarnitine analysis is included in newborn screening blood testing and is utilized for detection of several inborn errors of metabolism, including fatty acid oxidation disorders (FAOD) and organic acidemias (OA). For a 0.10 M solution of glutaric acid, HO2C(CH2)3CO2H (Ka1 = 4.6 × 10–5, Ka2 = 3.9 × 10–6), rank the following species in order of increasing equilibrium concentration It is found that the cocrystal physically decomposes at 120 °C (i.e., in the range between the melting points of the two pure compounds). Glutarex ®-2 Amino acid-modified medical food. Nutrition support of children and adults with glutaric aciduria type I. Blockage at GCDH leads to accumulation of glutaric acid and 3-hydroxyglutaric acid in blood and urine. The Ka value for most weak acids ranges from 10-2 to 10-14. Choosing an acid or base where pK a is close to the pH needed gives the best results. Find here Glutaric Acid, 110-94-1 manufacturers, suppliers & exporters in India. The molecular weight of Glutaric acid is 132.115 g/mol, while the boiling point of Glutaric acid is 579 ° F. Glutaric acid is partially soluble in water, and forms a moderately strong acid in water. 13 As with most organic acidurias and aminoacidopathies, the underlying pathophysiology includes intoxication with by‐products, namely glutaric acid and 3‐hydroxyglutaric acid, 10 leading to enzyme inhibition, substrate deficiencies and energy deficiency. Ka for HCN = 4.9 × 10–10 at 25°C. This work investigates the thermodynamics and crystallization of the theophylline–glutaric acid 1:1 cocrystal. 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