A Gas Chromatographic Procedure for Detection of Pathological Organic Aciduria

1970 ◽  
Vol 16 (3) ◽  
pp. 212-214 ◽  
Author(s):  
K B Hammond ◽  
S I Goodman
Keyword(s):  
Organic Acids ◽  
Carbon Disulfide ◽  
Dicarboxylic Acids ◽  
Short Chain Fatty Acids ◽  
Methylmalonic Aciduria ◽  
Organic Aciduria ◽  
Glycol Succinate ◽  
Glycol Adipate ◽  
Inborn Errors ◽  
Chromatographic Procedure

Abstract A number of nonamino organic acids associated with inborn errors of metabolism can be detected by gas chromatography. The organic acids are extracted into ethyl acetate and diethyl ether, the extracts combined, and the solvents evaporated under nitrogen. The residue is taken up in carbon disulfide and an aliquot chromatographed on 5% neopentyl glycol adipate to separate short-chain fatty acids (C 2 to 6). The remaining carbon disulfide is evaporated, the residue taken up in methanol, and the acids are methylated with diazomethane. The solvent is evaporated, the residue taken up in tetrahydrofuran, and an aliquot chromatographed on 15% diethylene glycol succinate to separate keto, hydroxy, and dicarboxylic acids. Chromatographic patterns for normal and abnormal urines have been established. Four cases of congenital methylmalonic aciduria have been diagnosed by this procedure.

METHYLMALONIC ACID

PEDIATRICS ◽  
1973 ◽  
Vol 51 (6) ◽  
pp. 1012-1015
Author(s):  
Lewis A. Barness
Keyword(s):  
Vitamin B12 ◽  
Inborn Errors Of Metabolism ◽  
Methylmalonic Acid ◽  
Methylmalonic Aciduria ◽  
Organic Aciduria ◽  
Combined System ◽  
Inborn Errors ◽  
System Disease ◽  
Amino Aciduria

Methylmalonate studies have led to some understanding of vitamin B12 metabolism as well as certain inborn errors of metabolism. These, in turn, have served as models of a group of diseases related to acidosis, so that the study of organic aciduria at present is similar to that of amino aciduria 20 years ago. Techniques for studying these have been developed. Many unanswered questions remain. (1) What does methylmalonate do? Does it, itself, cause the acidosis? Does it cause a deficiency of succinate in the oxidative cycle? (2) Are more direct ways of increasing succinate available? (3) What is the relation of methylmalonate to combined system disease or vitamin B12 neuropathy? (4) Are enzymes defective or absent? (5) What is the significance of methylmalonate in the newborn? (6) How does one counsel or treat families which include members with methylmalonic aciduria?

Use of a diode array detector in investigation of neonatal organic aciduria.

1985 ◽  
Vol 31 (4) ◽  
pp. 561-563 ◽  
Author(s):  
K R Allen ◽  
R Khan ◽  
D Watson
Keyword(s):  
Organic Acids ◽  
Methylmalonic Aciduria ◽  
Organic Aciduria ◽  
Array Detector ◽  
Diode Array ◽  
Diode Array Detection ◽  
Diode Array Detector ◽  
Maple Syrup ◽  
Urine Disease ◽  
Array Detection

Abstract We investigated the use of a diode array detector in conjunction with isocratic cation-exchange liquid chromatography for detection of organic acids in urine. The spectra and retention times of abnormal peaks found on chromatography of urine from patients with methylmalonic aciduria, maple syrup urine disease, and lactic aciduria were recorded and compared with those obtained for group of pure organic acids. Our results show that diode array detection helps considerably in tentatively identifying peaks appearing on an abnormal chromatogram while awaiting confirmation by mass spectrometry.

INFLUENCE OF DICARBOXYLIC ACIDS ON SOWING QUALITY OF SPRING BARLEY

1970 ◽  
pp. 22-25
Author(s):  
N. N. Loy ◽  
S. N. Gulina
Keyword(s):  
Organic Acids ◽  
Spring Barley ◽  
Dose Range ◽  
Dicarboxylic Acids ◽  
Rotary Machine ◽  
Working Solution ◽  
Negative Effect ◽  
Sprouted Seeds

The effect of presowing seed treatment on various concentrations of dicarboxylic (organic) acids on the sowing characteristics of spring barley has been studied. Seeds were treated with organic acids obtained by exposing cuttings to the radiation with a dose of 100 kGy and consequent hydrolysis, in concentrations: 1•10-7 %; 1•10-9; 1•10-11; 1•10-13 and 1•10-15 % on a laboratory rotary machine RVO-64 for one day before laying for germination. Distilled water was used for the control case. The rate of application of the working solution calculated as 10 liters / ton of seeds. Seeds were germinated in filter paper rolls in accordance with GOST 12038-84 requirements. The temperature was maintained at +24 ° C in the thermostat where the glasses with rolls were placed. For determination of germinative power and laboratory germination the sprouted seeds were evaluated after three and seven days, respectively. In laboratory experiments it was established that the treatment of barley seeds of varieties Zazersky 85, Nur and Vladimir with organic acids (OK) in different concentrations had both a stimulating and a negative effect. On the Zazersky 85 variety, in variants with acid concentrations of 1•10-9 and 1•10-11, an increase in germination energy (EP) by 2-4% and a significant decrease (by 3-4%) of laboratory germination (LV) of barley seeds were noted. On the Nur variety, the increase in EP was observed at 4% (concentration 1•10-11), LV and seed growth force (CPC) by 2-7% at a concentration of 1•10-7 and in the dose range 1•10-11 - 1•10-14 compared to the control values. On the grade of Vladimir, an increase in EP, LV, and CPC was found to increase by 1-6% at concentrations OK 1•10-7 and 1•10-13. It was shown that the treatment of seeds with acids led to an increase in the length of the germ in all studied varieties (by 3-9%) and dry biomass of 7-day-old seedlings - by 3-6%. Consequently, the treatment of seeds with a mixture of dicarboxylic acids has a stimulating effect on the sowing quality of spring barley.

Acrodermatitis-Like Syndrome in Organic Aciduria

PEDIATRICS ◽  
1994 ◽  
Vol 93 (3) ◽  
pp. 537-537
Author(s):  
U. Blecker ◽  
Y. Vandenplas ◽  
L. De Meirleir ◽  
L. De Raeve ◽  
J. Ramet
Keyword(s):  
Vitamin B12 ◽  
Autosomal Recessive ◽  
Clinical Manifestations ◽  
Methylmalonic Aciduria ◽  
Organic Aciduria ◽  
Asymptomatic Patients ◽  
High Doses

Methylmalonic aciduria (MMA) is an autosomal recessive in-born error of metabolism with a variation in the severity of the clinical manifestations, ranging from asymptomatic patients to fulminating neonatal forms causing severe ketosis, acidosis, hyperammonemia, pancytopenia, coma, and death. Severe cases can be treated with high doses of vitamin B12 and a diet low in proteins. We describe an exceptional manifestation of MMA. A 14-month-old boy with a neonatal manifestation of MMA was admitted during an intercurrent infection with ketoacidosis and hypoglycemia.

Prenatal Diagnosis of Methylmalonic Aciduria

PEDIATRICS ◽  
1974 ◽  
Vol 54 (4) ◽  
pp. 511-513
Author(s):  
D. Gompertz ◽  
Patricia A. Goodey ◽  
J. M. Saudubray ◽  
Christiane Charpentier ◽  
Agnes Chignolle ◽  
...  
Keyword(s):  
At Risk ◽  
Prenatal Diagnosis ◽  
Amniotic Fluid ◽  
Methylmalonic Acid ◽  
Methylmalonic Aciduria ◽  
Postnatal Period ◽  
Small Molecular Weight ◽  
Inborn Errors ◽  
Abnormal Accumulation ◽  
Amniotic Cell

The abnormal accumulation of small molecular weight metabolites in amniotic fluid in inborn errors of metabolism is unusual and prenatal diagnosis usually requires amniotic cell culture and specific enzyme assay. However, Morrow et al.1 reported raised concentrations of methylmalonic acid in the amniotic fluid of a pregnancy at risk from methylmalonic aciduria and confirmed the diagnosis in the postnatal period. More recently Mahoney et al.2 have reported an abnormal methylmalonic acid concentration in the amniotic fluid of one of two pregnancies at risk. They confirmed that the fetus in this case was affected by showing an impaired oxidation of methylmalonic acid in cultured amniotic cells.

Gas-chromatographic method of analysis for urinary organic acids. II. Description of the procedure, and its application to diagnosis of patients with organic acidurias.

1980 ◽  
Vol 26 (13) ◽  
pp. 1847-1853 ◽  
Author(s):  
K Tanaka ◽  
A West-Dull ◽  
D G Hine ◽  
T B Lynn ◽  
T Lowe
Keyword(s):  
Organic Acids ◽  
Chromatographic Method ◽  
Normal Subjects ◽  
Retention Indices ◽  
Organic Aciduria ◽  
Organic Acidurias ◽  
Urinary Organic Acids ◽  
Urinary Organic Acid Analysis ◽  
Gas Chromatographic Method ◽  
Extensive List

Abstract A gas-chromatographic method for urinary organic acid analysis is described, designed to be used routinely for the diagnosis of organic aciduria. It involves extraction of urine with ethyl acetate, dehydration of extract residues, and trimethylsilylation. Organic acids are identified by using an extensive list of retention indices published in the accompanying paper (this issue). Quantitative values are given for organic acids in urines from 50 ostensibly normal subjects. Typical chromatograms of urinary organic acids from patients with eight well-established organic acidurias are also shown.

Transport systems for organic acids induced in the marine pennate diatom, Cylindrotheca fusiformis

1972 ◽  
Vol 18 (2) ◽  
pp. 225-233 ◽  
Author(s):  
Johan A. Hellebust ◽  
Joyce Lewin
Keyword(s):  
Organic Acids ◽  
Dicarboxylic Acids ◽  
Metabolic Inhibitors ◽  
Transport Systems ◽  
Complex Relationship ◽  
Temperature Dependent ◽  
Pennate Diatom ◽  
Cylindrotheca Fusiformis ◽  
Uptake Rates ◽  
Marine Littoral

Cells of Cylindrotheca fusiformis, a marine littoral pennate diatom, can be induced to take up lactate by merely keeping them in the dark, and to take up succinate, fumarate, and malate as well as lactate by keeping them in the dark in the presence of any of these organic acids. Studies of uptake rates as a function of substrate concentration show a complex relationship indicating the presence of two or more uptake systems for any of these organic acids, each having different affinities and capacities for the substrates. Growth rate also shows a similar complex relationship to substrate concentration.Competition experiments indicate that lactate is taken up by different transport systems from those serving the 4-carbon dicarboxylic acids. Pyruvate is not taken up by C. fusiformis. Correlation of activities of enzymes involved in the immediate assimilation of the organic acids with the presence or absence of the ability to take up these substrates by cells incubated under different conditions show that the necessary assimilatory enzymes are already present and that it is rather the transport systems that are induced. The presence of glucokinase in dark-grown cells indicates that the inability of the cells to take up glucose is due to the lack of induction of a glucose transport system.The metabolic inhibitors dinitrophenol and iodoacetate inhibit the uptake of organic acids. However, since under normal conditions the organic acids are rapidly converted to other metabolites upon entry into the cells, and do not appear to accumulate, the observed inhibition may be at the level of assimilation rather than transport. The uptake of lactate and succinate is temperature dependent with a Q10 of 1.5–1.7, but is almost unaffected by changes in salinity from 10 to 150% of normal seawater. The relation between pH and rates of uptake of lactate and succinate suggests that the completely dissociated form of the organic acids is transported.

Thermal Properties of Ester-Type Easy Cationic Dyeable Poly(ethylene Terephthalate) (ECDP) Polymers

2010 ◽  
Vol 44-47 ◽  
pp. 2409-2413 ◽  
Author(s):  
Yu Sun ◽  
Guo Zheng ◽  
Hong Xiang Yang ◽  
Yan Jun Liu ◽  
Xiao Ning Li
Keyword(s):  
Thermal Properties ◽  
Diethylene Glycol ◽  
Ethylene Terephthalate ◽  
Glycol Succinate ◽  
Glycol Adipate ◽  
Scanning Calorimetry ◽  
Poly Ethylene Terephthalate ◽  
Monomer Content ◽  
Diethylene Glycol Succinate ◽  
Poly Ethylene

This study investigated the thermal properties of ester-type easy cationic dyeable poly(ethylene terephthalate) (ECDP) polymers using differential scanning calorimetry (DSC), therogravimetric analysis (TGA). The mass ratios of 5-sodium sulfo bis(-hydroxyethyl) isophthalate(SIPE) for ECDP polymers were 2.8%, 5.5%, 6.8%, respectively. The fourth monomers were diethylene glycol adipate (DGA), diethylene glycol succinate (DGS) and diethylene glycol subacate (DES) with different contents. The results suggested that the Tg of ester-type ECDP decreased with the increasing the molecule weight of the fourth monomer at fixed SIPE and fourth monomer contents. The Tch of ECDP polymer to be lower than that of the CDP polymer with the same SIPE content. And it decreased as SIPE and fourth monomer contents increased, it also decreased with the increasing of the molecule weight of the fourth monomer given the same SIPE content. The effect of the ester-type soft segments reduced the Tm of ECDP. The thermal stability of ECDP polymer was less than PET and CDP polymers, and it decreased with increasing SIPE content, but increased with the ester-type fourth monomer content increasing.

scholarly journals Vertical distribution of particle-phase dicarboxylic acids, oxoacids and α-dicarbonyls in the urban boundary layer based on the 325-meter tower in Beijing

2020 ◽  
Author(s):  
Wanyu Zhao ◽  
Hong Ren ◽  
Kimitaka Kawamura ◽  
Huiyun Du ◽  
Xueshun Chen ◽  
...  
Keyword(s):  
Boundary Layer ◽  
Oxalic Acid ◽  
Vertical Distribution ◽  
Organic Acids ◽  
Anthropogenic Activities ◽  
Ground Surface ◽  
Dicarboxylic Acids ◽  
Ground Level ◽  
Water Soluble ◽  
Motor Vehicles

Abstract. Vertical distribution of dicarboxylic acids, oxoacids, α-dicarbonyls, and other organic tracer compounds in fine aerosols (PM2.5) was investigated from the ground surface (8 m) to 260 m at a 325-meter meteorological tower in Beijing in the summer of 2015. Results showed that the concentrations of oxalic acid (C2), the predominant diacid, were more abundant at 120 m (210 ± 154 ng m−3) and 260 m (220 ± 140 ng m−3) than those at the ground level (160 ± 90 ng m−3). Concentrations of phthalic acid (Ph) decreased with the increase of heights, demonstrating that the vehicular exhausts at the ground surface was the main contributor. Positive correlations were noteworthy for C2/total diacids with mass ratios of C2 to main oxoacids (Pyr, ωC2) and α-dicarbonyls (Gly, MeGly) in polluted days (0.42 ≤ r2 ≤ 0.65), especially at the ground level. In clean days, the ratios of carbon content in oxalic acid to water soluble organic carbon (C2-C/WSOC) showed larger values at 120 m and 260 m than those at the ground surface. However, in polluted days, the C2-C/WSOC ratio mainly reached its maximum at the ground level. These phenomena may indicate the enhanced contribution of aqueous-phase oxidation to oxalic acid in polluted days. Combined with the influence of wind field, total diacids, oxoacids and α-dicarbonyls decreased by 22 %–58 % under the control on anthropogenic activities during the 2015 Victory Parade period. Furthermore, the PMF results showed that the secondary formation routes (secondary sulfate formation and secondary nitrate formation) were the dominant contributors (37–44 %) to organic acids, followed by biomass burning (25–30 %) and motor vehicles (18–24 %). In this study, the organic acids at the ground level were largely associated with local traffic emissions, while the long-range atmospheric transport followed by photochemical aging contributed more to diacids and related compounds in the boundary layer over Beijing than the ground surface.

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