Acylglycine Analysis by Ultra‐Performance Liquid Chromatography‐Tandem Mass Spectrometry (UPLC‐MS/MS)

Hobert, Judith A.; Liu, Aiping; Pasquali, Marzia

doi:10.1002/cphg.19

Cited by 9 publications

(5 citation statements)

References 21 publications

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“…Phenylpropionylglycine is a specific marker for MCAD, although it may not be detected if the patient is undergoing antibiotic therapy, or in neonates and young infants, since it is an intestinal bacterial product. New tandem mass spectrometric methods are very useful for increasing the detection and recovery of this acylglycine [ 70 ]. Acylglycines are also excreted following MCT supplementation, especially suberylglycine [ 27 , 71 ].…”

Section: Study Of Metabolites In the Diagnosis Of Fatty Acid Oxidation Defectsmentioning

confidence: 99%

Biochemical Markers for the Diagnosis of Mitochondrial Fatty Acid Oxidation Diseases

Ruíz-Sala

Quintana

2021

JCM

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Mitochondrial fatty acid β-oxidation (FAO) contributes a large proportion to the body’s energy needs in fasting and in situations of metabolic stress. Most tissues use energy from fatty acids, particularly the heart, skeletal muscle and the liver. In the brain, ketone bodies formed from FAO in the liver are used as the main source of energy. The mitochondrial fatty acid oxidation disorders (FAODs), which include the carnitine system defects, constitute a group of diseases with several types and subtypes and with variable clinical spectrum and prognosis, from paucisymptomatic cases to more severe affectations, with a 5% rate of sudden death in childhood, and with fasting hypoketotic hypoglycemia frequently occurring. The implementation of newborn screening programs has resulted in new challenges in diagnosis, with the detection of new phenotypes as well as carriers and false positive cases. In this article, a review of the biochemical markers used for the diagnosis of FAODs is presented. The analysis of acylcarnitines by MS/MS contributes to improving the biochemical diagnosis, both in affected patients and in newborn screening, but acylglycines, organic acids, and other metabolites are also reported. Moreover, this review recommends caution, and outlines the differences in the interpretation of the biomarkers depending on age, clinical situation and types of samples or techniques.

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Section: Study Of Metabolites In the Diagnosis Of Fatty Acid Oxidation Defectsmentioning

confidence: 99%

Biochemical Markers for the Diagnosis of Mitochondrial Fatty Acid Oxidation Diseases

Ruíz-Sala

Quintana

2021

JCM

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“…The urinary acylglycine was butylated according to the method of Hobert et al. ( 15 ) & Fisher et al. ( 16 ) and analyzed by LC tandem MS (TSQ Altis, Thermo Scientific).…”

Section: Methodsmentioning

confidence: 99%

“…15 N-hexanoylglycine, 2 H 2 -octanoylglycine, 2 H 2 -bezoylglycine) used were from Cambridge Isotope Laboratories Inc. The urinary acylglycine was butylated according to the method of Hobert et al(15) & Fisher et al (16) and analyzed by LC tandem MS (TSQ Altis, Thermo Scientific).…”

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confidence: 99%

The impact of obesity-associated glycine deficiency on the elimination of endogenous and exogenous metabolites via the glycine conjugation pathway

Tan,

Hsu,

Tai

et al. 2024

Front. Endocrinol.

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BackgroundGlycine is an integral component of the human detoxification system as it reacts with potentially toxic exogenous and endogenously produced compounds and metabolites via the glycine conjugation pathway for urinary excretion. Because individuals with obesity have reduced glycine availability, this detoxification pathway may be compromised. However, it should be restored after bariatric surgery because of increased glycine production.ObjectiveTo examine the impact of obesity-associated glycine deficiency on the glycine conjugation pathway. We hypothesize that the synthesis rates of acylglycines from endogenous and exogenous sources are significantly reduced in individuals with obesity but increase after bariatric surgery.MethodsWe recruited 21 participants with class III obesity and 21 with healthy weight as controls. At baseline, [1,2-13C2] glycine was infused to study the glycine conjugation pathway by quantifying the synthesis rates of several acylglycines. The same measurements were repeated in participants with obesity six months after bariatric surgery. Data are presented as mean ± standard deviation, and p-value< 0.05 is considered statistically significant.ResultsBaseline data of 20 participants with obesity were first compared to controls. Participants with obesity were significantly heavier than controls (mean BMI 40.5 ± 7.1 vs. 20.8 ± 2.1 kg/m2). They had significantly lower plasma glycine concentration (168 ± 30 vs. 209 ± 50 μmol/L) and slower absolute synthesis rates of acetylglycine, isobutyrylglycine, tigylglycine, isovalerylglycine, and hexanoylglycine. Pre- and post-surgery data were available for 16 participants with obesity. Post-surgery BMI decreased from 40.9 ± 7.3 to 31.6 ± 6.0 kg/m2. Plasma glycine concentration increased from 164 ± 26 to 212 ± 38 μmol/L) and was associated with significantly higher rates of excretion of acetylglycine, isobutyrylglycine, tigylglycine, isovalerylglycine, and hexanoylglycine. Benzoic acid (a xenobiotic dicarboxylic acid) is excreted as benzoylglycine; its synthesis rate was significantly slower in participants with obesity but increased after bariatric surgery.ConclusionObesity-associated glycine deficiency impairs the human body’s ability to eliminate endogenous and exogenous metabolites/compounds via the glycine conjugation pathway. This impairment is ameliorated when glycine supply is restored after bariatric surgery. These findings imply that dietary glycine supplementation could treat obesity-associated metabolic complications due to the accumulation of intramitochondrial toxic metabolites.Clinical trial registrationhttps://clinicaltrials.gov/study/NCT04660513, identifier NCT04660513.

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“…8 In addition to GC−MS analysis, LC−MS has also been applied for the analysis of NAGlys for a long time. 9,10 Their direct detection in urine samples has been investigated. 11 However, owing to the wide polarity of NAGlys, it is hard to quantify NAGlys with high and low polarity simultaneously and accurately.…”

Section: ■ Introductionmentioning

confidence: 99%

Derivatization of N-Acyl Glycines by 3-Nitrophenylhydrazine for Targeted Metabolomics Analysis and Their Application to the Study of Diabetes Progression in Mice

Shi

et al. 2023

Anal. Chem.

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N-Acyl glycines (NAGlys) are an important class of metabolites in the detoxification system of the human body. They have been used in the diagnosis of several metabolic diseases. Liquid chromatography−mass spectrometry (LC−MS) is the most frequently used NAGlys detection platform. Here, we describe a simple and sensitive method of NAGlys detection by LC−MS in plasma and urine samples. This approach is based on the use of a derivatization reagent, 3-nitrophenylhydrazine. The reaction is quick in aqueous solution, and no quenching step is needed. To expand the coverage of NAGlys when standards are not available, NAGlys were first identified based on high-resolution LC−MS. Quantification was subsequently carried out on triple quadrupole LC−MS. This approach allowed a much broader measurement of NAGlys (41 NAGlys in total), especially when authentic standards are unavailable. Comprehensive analysis of NAGlys with this new method was applied in plasma and urine samples of db/db diabetic and non-diabetic db/m+ control mice. The majority of detected NAGlys were altered with high differentiation ability in plasma and urine samples from diabetic and non-diabetic mice. These identified NAGlys hold the potential to be diagnostic biomarkers for type II diabetes and diabetic complications.

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Acylglycine Analysis by Ultra‐Performance Liquid Chromatography‐Tandem Mass Spectrometry (UPLC‐MS/MS)

Cited by 9 publications

References 21 publications

Biochemical Markers for the Diagnosis of Mitochondrial Fatty Acid Oxidation Diseases

Biochemical Markers for the Diagnosis of Mitochondrial Fatty Acid Oxidation Diseases

The impact of obesity-associated glycine deficiency on the elimination of endogenous and exogenous metabolites via the glycine conjugation pathway

Derivatization of N-Acyl Glycines by 3-Nitrophenylhydrazine for Targeted Metabolomics Analysis and Their Application to the Study of Diabetes Progression in Mice

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