Neutral aminoaciduria in cystathionine β-synthase-deficient mice, an animal model of homocystinuria

2014 ◽  
Vol 306 (12) ◽  
pp. F1462-F1476 ◽  
Author(s):  
Noriyuki Akahoshi ◽  
Shotaro Kamata ◽  
Masashi Kubota ◽  
Takako Hishiki ◽  
Yoshiko Nagahata ◽  
...  
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Serum Levels ◽  
Substrate Affinity ◽  
Neutral Amino Acids ◽  
Amino Acid Profiles ◽  
Marked Accumulation ◽  
Urinary Amino ◽  
Severe Growth ◽  
Deficient Mice

The kidney is one of the major loci for the expression of cystathionine β-synthase (CBS) and cystathionine γ-lyase (CTH). While CBS-deficient ( Cbs−/−) mice display homocysteinemia/methioninemia and severe growth retardation, and rarely survive beyond the first 4 wk, CTH-deficient ( Cth−/−) mice show homocysteinemia/cystathioninemia but develop with no apparent abnormality. This study examined renal amino acid reabsorption in those mice. Although both 2-wk-old Cbs−/− and Cth−/− mice had normal renal architecture, their serum/urinary amino acid profiles largely differed from wild-type mice. The most striking feature was marked accumulation of Met and cystathionine in serum/urine/kidney samples of Cbs−/− and Cth−/− mice, respectively. Levels of some neutral amino acids (Val, Leu, Ile, and Tyr) that were not elevated in Cbs−/− serum were highly elevated in Cbs−/− urine, and urinary excretion of other neutral amino acids (except Met) was much higher than expected from their serum levels, demonstrating neutral aminoaciduria in Cbs−/− (not Cth−/−) mice. Because the bulk of neutral amino acids is absorbed via a B0AT1 transporter and Met has the highest substrate affinity for B0AT1 than other neutral amino acids, hypermethioninemia may cause hyperexcretion of neutral amino acids.

scholarly journals A Specific Urinary Amino Acid Profile Characterizes People with Kidney Stones

2020 ◽  
Vol 2020 ◽  
pp. 1-7 ◽  
Author(s):  
Aniello Primiano ◽  
Silvia Persichilli ◽  
Pietro Manuel Ferraro ◽  
Riccardo Calvani ◽  
Alessandra Biancolillo ◽  
...  
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Kidney Stones ◽  
Stone Formation ◽  
Amino Acid Profile ◽  
Stone Formers ◽  
Stone Former ◽  
Amino Acid Profiles ◽  
Definition Of ◽  
Urinary Amino

Background. Urolithiasis is the process of stone formation in the urinary tract. Its etiology is only partly known, and efficient therapeutic approaches are currently lacking. Metabolomics is increasingly used in biomarkers discovery for its ability to identify mediators of relevant (patho)physiological processes. Amino acids may be involved in kidney stone formation. The aim of the present study was to investigate the presence of an amino acid signature in stone former urine through a targeted metabolomic approach. Methods. A panel of 35 amino acids and derivatives was assessed in urines from 15 stone former patients and 12 healthy subjects by UPLC-MS. Partial Least Squares Discriminant Analysis (PLS-DA) was used to define amino acid profiles of cases and controls. Results and Discussion. Our approach led to the definition of a specific amino acid fingerprint in people with kidney stones. A urinary amino acid profile of stone formers was characterized by lower levels of α-aminobutyric acid, asparagine, ethanolamine, isoleucine, methionine, phenylalanine, serine, tryptophan, and valine. Metabolomic analysis may lend insights into the pathophysiology of urolithiasis and allow tracking this prevalent condition over time.

Sex-Related Patterns in the Profiles of Human Urinary Amino Acids

1975 ◽  
Vol 21 (13) ◽  
pp. 1970-1975 ◽  
Author(s):  
Henri Dirren ◽  
Arthur B Robinson ◽  
Linus Pauling
Keyword(s):  
Amino Acids ◽  
High Pressure ◽  
Amino Acid ◽  
Young Women ◽  
Amino Acid Analyzer ◽  
Amino Acid Profiles ◽  
Urinary Amino Acids ◽  
Urinary Amino ◽  
Nonparametric Statistical ◽  
Related Pattern

Abstract The urinary amino acid profiles of 79 young women and 72 young men were studied with an automated high-pressure amino acid analyzer. A nonparametric statistical analysis was applied for detecting profile differences related to sex and other variables. A strong sex-related pattern was confirmed and its power for sex determination was evaluated. For the women, profile differences related to the use of oral contraceptives were also discovered and evaluated. No correlation with the menstrual cycle was detected.

scholarly journals Amino Acid and Acylcarnitine Levels in Chronic Patients with Schizophrenia: A Preliminary Study

Metabolites ◽  
2021 ◽  
Vol 11 (1) ◽  
pp. 34
Author(s):  
Irina A. Mednova ◽  
Alexander A. Chernonosov ◽  
Marat F. Kasakin ◽  
Elena G. Kornetova ◽  
Arkadiy V. Semke ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Amino Acids ◽  
Amino Acid ◽  
Chronic Schizophrenia ◽  
Integrated Approach ◽  
Serum Levels ◽  
Tandem Mass ◽  
Chronic Patients ◽  
Healthy Donors ◽  
Preliminary Study

Amino acids and acylcarnitines play an important role as substrates and intermediate products in most of pathways involved in schizophrenia development such as mitochondrial dysfunction, inflammation, lipid oxidation, DNA damage, oxidative stress, and apoptosis. It seems relevant to use an integrated approach with ‘omics’ technology to study their contribution. The aim of our study was to investigate serum amino acid and acylcarnitine levels in antipsychotics-treated patients with chronic schizophrenia compared with healthy donors. We measured serum levels of 15 amino acids and 30 acylcarnitines in 37 patients with schizophrenia and 36 healthy donors by means of tandem mass spectrometry. In summary, patients with chronic schizophrenia had an altered concentration of a few amino acids and acylcarnitines in comparison to the healthy probands. Further research is needed to assess and understand the identified changes.

scholarly journals Changes in Amino Acid Profiles and Bioactive Compounds of Thai Silk Cocoons as Affected by Water Extraction

Molecules ◽  
2021 ◽  
Vol 26 (7) ◽  
pp. 2033
Author(s):  
Chuleeporn Bungthong ◽  
Sirithon Siriamornpun
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Biological Activities ◽  
Radical Scavenging Activity ◽  
Radical Scavenging ◽  
Silk Protein ◽  
Extraction Time ◽  
Antioxidant Power ◽  
Silk Cocoon ◽  
Amino Acid Profiles

Silk proteins have many advantageous components including proteins and pigments. The proteins—sericin and fibroin—have been widely studied for medical applications due to their good physiochemical properties and biological activities. Various strains of cocoon display different compositions such as amino-acid profiles and levels of antioxidant activity. Therefore, the objectives of this study were to find a suitable silk protein extraction method to obtain products with chemical and biological properties suitable as functional foods in two strains of Bombyx mori silk cocoon (Nangsew strains; yellow cocoon) and Samia ricini silk cocoon (Eri strains; white cocoon) extracted by water at 100 °C for 2, 4, 6 and 8 h. The results showed that Nangsew strains extracted for 6 h contained the highest amounts of protein, amino acids, total phenolics (TPC) and total flavonoids (TFC), plus DPPH radical-scavenging activity, ABTS radical scavenging capacity, and ferric reducing antioxidant power (FRAP), anti-glycation, α-amylase and α-glucosidase inhibition. The longer extraction time produced higher concentrations of amino acids, contributing to sweet and umami tastes in both silk strains. It seemed that the bitterness decreased as the extraction time increased, resulting in improvements in the sweetness and umami of silk-protein extracts.

scholarly journals Serum Amino Acid Profiles in Childhood Predict Triglyceride Level in Adulthood: A 7-Year Longitudinal Study in Girls

2016 ◽  
Vol 101 (5) ◽  
pp. 2047-2055 ◽  
Author(s):  
Petri Wiklund ◽  
Xiaobo Zhang ◽  
Xiao Tan ◽  
Sirkka Keinänen-Kiukaanniemi ◽  
Markku Alen ◽  
...  
Keyword(s):  
Amino Acids ◽  
Longitudinal Study ◽  
Amino Acid ◽  
High Risk ◽  
Triglyceride Level ◽  
Early Adulthood ◽  
Health Science ◽  
Model Assessment ◽  
Pubertal Growth ◽  
Amino Acid Profiles

AbstractContext:Branched-chain and aromatic amino acids are associated with high risk of developing dyslipidemia and type II diabetes in adults.Objective:This study aimed to examine whether serum amino acid profiles associate with triglyceride concentrations during pubertal growth and predict hypertriglyceridemia in early adulthood.Design:This was a 7.5-year longitudinal study.Setting:The study was conducted at the Health Science Laboratory, University of Jyväskylä.Participants:A total of 396 nondiabetic Finnish girls aged 11.2 ± 0.8 years at the baseline participated in the study.Main Outcome Measures:Body composition was assessed by dual-energy x-ray absorptiometry; serum concentrations of glucose, insulin, and triglyceride by enzymatic photometric methods; and amino acids by nuclear magnetic resonance spectroscopy.Results:Serum leucine and isoleucine correlated significantly with future triglyceride, independent of baseline triglyceride level (P < .05 for all). In early adulthood (at the age of 18 years), these amino acids were significantly associated with hypertriglyceridemia, whereas fat mass and homeostasis model assessment of insulin resistance were not. Leucine was the strongest determinant discriminating subjects with hypertriglyceridemia from those with normal triglyceride level (area under the curve, 0.822; 95% confidence interval, 0.740–0.903; P = .000001).Conclusions:Serum leucine and isoleucine were associated with future serum triglyceride levels in girls during pubertal growth and predicted hypertriglyceridemia in early adulthood. Therefore, these amino acid indices may serve as biomarkers to identify individuals at high risk for developing hypertriglyceridemia and cardiovascular disease later in life. Further studies are needed to elucidate the role these amino acids play in the lipid metabolism.

scholarly journals 131 MODIFICATION OF AMINO ACID CONCENTRATIONS IN MEDIUM BY PIG EMBRYOS FROM THE ZYGOTE TO THE BLASTOCYST STAGE

2005 ◽  
Vol 17 (2) ◽  
pp. 216
Author(s):  
P. Booth ◽  
T. Watson ◽  
H. Leese
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Embryonic Development ◽  
Amino Acid Profile ◽  
Blastocyst Stage ◽  
Morula Stage ◽  
Amino Acid Profiles ◽  
The Difference ◽  
Amino Acid Concentrations

Pre-implantation embryos can produce and consume amino acids in a manner dependent upon stage of embryonic development (Partridge and Leese 1996 Reprod. Fert. Dev. 8, 945) that may also be predictive of subsequent viability (Houghton et al. 2002 Hum. Reprod. 17, 999). To examine these relationships in the pig, the appearance or depletion of 18 amino acids from a presumptive near-physiological mixture was determined by HPLC in porcine in vitro-produced embryos from the zygote to the blastocyst stage. Cumulus oocyte complexes derived from slaughterhouse prepubertal pig ovaries were matured for 40 h in modified TCM-199 before being fertilized (Day 0) with frozen thawed semen in tris-based medium. After 6 h, presumptive zygotes were denuded and cultured in groups of 20 in NCSU medium modified to contain a physiological mixture of 18 amino acids including 0.1 mM glutamine (NCSUaa). Groups of 2–10 embryos (dependent on stage) were removed on Day 0 (1 cell), Day 1 (2- and 4-cell), Day 4 (compact morula), and Day 6 (blastocyst) and placed in 4 μL NCSUaa for 24 h. After incubation, the embryos were removed and the medium analyzed by HPLC. Each stage was replicated 3–9 times. Since amino acid profiles of 2- and 4-cell embryos were not different, data were combined. Overall, arginine (1.19 ± 0.33), glutamine (0.78 ± 0.34) and threonine (0.05 ± 0.04) were significantly (P < 0.01) depleted from the medium whereas alanine (0.21 ± 0.1), glycine (0.20 ± 0.06), asparagine (0.13 ± 0.5), lysine (0.1 ± 0.03), isoleucine (0.08 ± 0.01), valine (0.05 ± 0.01), leucine (0.04 ± 0.02), phenylalanine (0.03 ± 0.01), and histidine (0.02 ± 0.04) significantly (P < 0.05) accumulated (mean of the 4 sampling timepoints; all values pmol/embryo/h ± SEM). The difference between amino acid accumulation and depletion (balance) was approximately equivalent between Day 0 and the morula stage although turnover (sum of depletion and accumulation) steadily decreased during this period from 3.1 on Day 0 to 1.35 pmol/embryo/h at the morula stage. However, at the blastocyst stage, turnover and balance increased to 6.32 and 2.42 pmol/embryo/h, respectively, i.e. net appearance occurred. Notable changes in amino acid profile during development included decreases in accumulation of asparagine, glutamate, and glycine in the medium and the depletion of glutamine over Days 0, 1, and 4, followed by reversal of these trends by Day 6. These data suggest that pig embryos can alter the accumulation and depletion rates of amino acids in a manner that is dependent on the specific amino acid and the stage of embryonic development. This work was supported by BBSRC.

AMINO ACIDS IN THE BLOOD AND URINE OF NORMAL AND ARTHRITIC SUBJECTS BEFORE AND AFTER A GLYCINE LOAD GIVEN WITH AND WITHOUT ADRENOCORTICOTROPIN

1957 ◽  
Vol 35 (1) ◽  
pp. 1005-1016 ◽  
Author(s):  
J. B. Derrick ◽  
Audrey P. Hanley
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Chromatographic Analysis ◽  
Free Amino ◽  
Serum Levels ◽  
Essential Amino Acids ◽  
Single Amino Acid ◽  
Normal Individuals ◽  
Before And After ◽  
Serum And Urine

Observations have been made on the specific free amino acids (chromatographic analysis) and other nitrogenous constituents in the serum and urine of normal and arthritic men under controlled dietary conditions, before and after a glycine load and adrenocorticotropin administered separately and together.Differences in the metabolism of amino acids between normal individuals and arthritics, particularly of alanine, proline, glutamic acid, taurine, and possibly tyrosine (and/or tryptophan) and cystine, were apparent. The differences were largely confined to the non-essential amino acids. Concomitant increases seen in the serum levels and in the excretion of several amino acids, in response to a load of a single amino acid, indicate that the increases in excretion are more than a matter of competition for reabsorption in the kidney. A prerenal phenomenon appears to be involved, possibly interconversion of amino acids. This concept is supported by the evidence that the increases in the serum levels were restricted to the non-essential amino acids.

AMINO ACIDS IN THE BLOOD AND URINE OF NORMAL AND ARTHRITIC SUBJECTS BEFORE AND AFTER A GLYCINE LOAD GIVEN WITH AND WITHOUT ADRENOCORTICOTROPIN

1957 ◽  
Vol 35 (11) ◽  
pp. 1005-1016 ◽  
Author(s):  
J. B. Derrick ◽  
Audrey P. Hanley
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Chromatographic Analysis ◽  
Free Amino ◽  
Serum Levels ◽  
Essential Amino Acids ◽  
Single Amino Acid ◽  
Normal Individuals ◽  
Before And After ◽  
Serum And Urine

Observations have been made on the specific free amino acids (chromatographic analysis) and other nitrogenous constituents in the serum and urine of normal and arthritic men under controlled dietary conditions, before and after a glycine load and adrenocorticotropin administered separately and together.Differences in the metabolism of amino acids between normal individuals and arthritics, particularly of alanine, proline, glutamic acid, taurine, and possibly tyrosine (and/or tryptophan) and cystine, were apparent. The differences were largely confined to the non-essential amino acids. Concomitant increases seen in the serum levels and in the excretion of several amino acids, in response to a load of a single amino acid, indicate that the increases in excretion are more than a matter of competition for reabsorption in the kidney. A prerenal phenomenon appears to be involved, possibly interconversion of amino acids. This concept is supported by the evidence that the increases in the serum levels were restricted to the non-essential amino acids.

Dissociation between plasma and brain amino acid profiles and short-term food intake in the rat

1994 ◽  
Vol 266 (5) ◽  
pp. R1675-R1686 ◽  
Author(s):  
G. H. Anderson ◽  
E. T. Li ◽  
S. P. Anthony ◽  
L. T. Ng ◽  
R. Bialik
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Food Intake ◽  
Amino Acid Mixture ◽  
Acid Mixture ◽  
Short Term ◽  
Egg Albumin ◽  
Amino Acid Profiles ◽  
Total Mixture ◽  
Brain Amino Acid

The relationship between plasma and brain amino acids and short-term food intake after administration of albumin, or its constituent amino acids, was examined. Rats given protein (0.85 g chicken egg albumin) or an amino acid mixture patterned after egg albumin reduced their food intake during 1 h of feeding beginning 30 min after gavage. Similarly, when given separately, the essential (EAA) and nonessential amino acid (NEAA) fractions of egg albumin caused comparable decreases in food intake. As the dose increased from 0.5 to 1.5 g the duration of anorexia prolonged to 12 h. Little change occurred in plasma amino acids at 30 and 60 min after albumin at 0.85 g, although many increased by 25-50% at 60 min after 1.5 g. Marked changes in plasma occurred after gavage with the total mixture of constituent free amino acids and after either EAA or NEAA fractions. Brain amino acid concentrations were little affected by albumin and did not show consistent changes after the amino acid treatments. Thus the reductions in food intake after ingestion of albumin or of its constituent amino acids were not predicted from the resulting changes in either plasma or brain concentrations of amino acids.

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