Urine succinic acid

Defects of the Krebs cycle. Fumarase deficiency was reported in children with mitochondrial encephalomyop-athy. Usually, there is developmental delay since early infancy, microcephaly, hypotonia and cerebral atrophy, with death in infancy or early childhood. The laboratory hallmark of the disease is the excretion of large amounts of fumaric acid and, to a lesser extent, succinic acid in the urine. The enzyme defect has been found in muscle, liver and cultured skin fibroblasts [16]. 

Figure 4.10 Direct analysis of catecholamines in urine sample. Column, Asahipak ES-502C eluent, 75 mM succinic acid + 25 mM borate buffer (pH 6.10) containing 0.5 mM EDTA flow rate, 1.0 min-1 detection, fluorescence reaction detection Ex. 350 nm. Peaks-. 1, adrenaline-, 2, noradrenaline-, and 3, dopamine.

Giordano G, McMurray WJ, Previs SL, Welch RD, Rinaldo P (1990) Identification of 2-(2 -octenyl) succinic acid in urine. Rapid Commun Mass Spectrom 4 170-172... 

Disposition in the Body. Slowly and incompletely absorbed after oral administration absorbed after intramuscular administration but generally given by the intravenous route. Rapidly distributed into the extracellular fluids throughout the body. Suxamethonium is rapidly hydrolysed in plasma and body tissues to succinylmonocholine (weak activity) and choline. Succinylmonocholine is then slowly hydrolysed to succinic acid and choline. Less than 3% of a dose is excreted unchanged in the urine. 

As a result of GC-MS analyses, 103 metabolites were determined, of which 66 were successfully identified and 18 were used to create a diagnostic model. Of these 18 metabolites, 5 (suberic acid, glycine, L-tyrosine, L-threonine, and succinic acid) had significantly higher levels in patients with HCC than in healthy volunteers (p < 0.05). Other metabolites (oxalic acid, xylitol, urea, phosphates, propanoic acid, threonine, pimelic acid, butyric acid, trihydroxypentanoic acid, hypoxanthine, arabinofuranose, dipeptide of hydroxyproline, and tetrahydroxypentanoic acid) showed higher levels in healthy volunteers (p < 0.05). In addition, Wu et al. determined the levels of AFP using an ELISA test in serum from the same patients and healthy volunteers as in the metabolomic study of urine samples. An AFP concentration above 20 ng/mL suggests a positive result and the presence of... 

Historical Development. Citric acid was the first of these acids to be identified in blood and urine. A few studies were later concerned with a-ketoglutaric and succinic acids in these biological fluids, until the development of chromatographic techniques allowed the determination of all acids of the tricarboxylic acid cycle, except the unstable ones (oxa-losuccinic and oxalacetic acids). Besides the chromatographic techniques, new enzymatic and fluorimetric methods have been described for some of these acids, including oxalacetic acid. 

S. a,a-Dimethylsuccinic Acid. First found, like methylmalonic acid, in the urine of rats fed a necrogenic diet (F6), a,a-dimethyl-succinic acid was identified later in the urine of normal rats (B3). Its origin is unknown,... 

A previously unknown concrete acid calculus) was separated by Scheele from urinary calculi (uric acid) and shown to be present in small quantities in urine. The red colour (murexide) formed on evaporating it with nitric acid was observed. By heating uric acid he obtained a sublimate (afterwards called pyrouric acid) which he thought was similar to succinic acid it is really cyanuric acid. ... 

Vitamin B,2 serves as the coenzyme for some enzymes. One of these is the enzyme that catalyzes the reaction of isomerization of methylmalonic acid, H00CCH(CH3)C00H, to succinic acid. Some children suffer from the disease methylmalonicaciduria, in which the concentration of methylmalonic acid in the blood and urine is very large because of a genetic defect that causes an abnormality in this enzyme and the consequent failure to convert the methylmalonic acid to succinic acid. For about half of the patients the disease can be kept under control by the daily intake of about 1 mg of B12, 500 times the usually recommended intake. The probable explanation of this effect is that the patient manufactures a defective apoenzyme with a very small combination constant with the coenzyme, so that at equilibrium under ordinary nutrition only a very small fraction of the apoenzyme is converted to the active enzyme. It can be seen from Equation 14-4, however, that the amount converted to active enzyme is increased by an increase in the concentration of the coenzyme. 

Disorders of GABA Vitamin B6-dependent seizures Often an absence of succinic semialdehyde dehydrogenase Hypotonia, ataxia, mental retardation in older child. Increased urine 4-OH-butyric acid. Pyridoxine (B6-dependent disorder) Inhibitors of GABA transaminase... 

The initial metabolic fate of (Z,Z)-muconic acid in bacteria is enzyme-catalyzed ring closure to muconolactone (37) [43] (see Scheme 4) en route to succinate and acetyl CoA. The data in Table 1 indicate that this reaction is exothermic by ca. 12 kcal/mol. This simple ring closure is, of course, not available to the ( , )-isomer some of which is found in mammalian urine, although metabolism of muconic acid yields some CO2 [/]. 

You are examining mitochondria from muscle cells of an infant who has a deficiency in one of the enzymes in the fatty acid oxidative pathway. The mitochondria consume oxygen normally when incubated with pyruvate and malate, "with succinate, or with palmitoyl CoA (in the presence of carnitine), but the rate of oxygen utilization is decreased when the mitochondria are incubated with linoleoyl CoA in the presence of carnitine. Blood levels of carnitine in the patient are low, while the levels of an unusual acylcami-tine derivative are present in blood and urine. Analysis of this acylcamitine species using mass spectroscopy reveals that it is trans-A, cis-A decadienoyl (C10 2)-acylcamitine. The infant suffers from hypotonia (lack of muscle tone) and slow weight gain. 

The alchemists and early chemists often worked with materials such as blood, saliva, urine, egg-white, gum, etc. One of the first organic compounds obtained in a fairly pure state was alcohol (ethanol). It was prepared by distillation in Europe in the twelfth century, and it may also have been familiar to the Arab alchemists. Ethanol had also been obtained many centuries earlier in China, both by distillation and by removing the water from a dilute solution by freezing. In the sixteenth century, the products of the reaction of alcohol with each of the three mineral acids had been prepared. Before 1780 only four organic acids were known formic, acetic, benzoic, and succinic. Around this time, Scheele isolated several more. 

These compounds, occuring mostly in urine or obtained synthetically, are derivatives of androgens, corticosteroid metabolites and oestrogens they are principally sulphate or phosphate esters or glucuronides (combined with glucuronic acid via a jff-glycosidic link). Their separation in column or paper chromatography has been described earlier (cf. [129]). Synthetic acid esters of steroids with succinic or tetrahydrophthahc acids may be classified here also. 

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