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Homocystine

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https://www.readbyqxmd.com/read/28343144/gene-and-protein-expressions-and-metabolomics-exhibit-activated-redox-signaling-and-wnt-%C3%AE-catenin-pathway-are-associated-with-metabolite-dysfunction-in-patients-with-chronic-kidney-disease
#1
Dan-Qian Chen, Gang Cao, Hua Chen, Dan Liu, Wei Su, Xiao-Yong Yu, Nosratola D Vaziri, Xiu-Hua Liu, Xu Bai, Li Zhang, Ying-Yong Zhao
Changes in plasma concentration of small organic metabolites could be due to their altered production or urinary excretion and changes in their urine concentration may be due to the changes in their filtered load, tubular reabsorption, and/or altered urine volume. Therefore, these factors should be considered in interpretation of the changes observed in plasma or urine of the target metabolite(s). Fasting plasma and urine samples from 180 CKD patients and 120 age-matched healthy controls were determined by UPLC-HDMS-metabolomics and quantitative real-time RT-PCR techniques...
March 23, 2017: Redox Biology
https://www.readbyqxmd.com/read/28271225/development-of-a-matrix-based-candidate-reference-material-of-total-homocysteine-in-human-serum
#2
Yu Liu, Dewei Song, Bei Xu, Hongmei Li, Xinhua Dai, Baorong Chen
We developed and evaluated a candidate serum reference material to help improve clinical routine measurement, and to provide traceability of the measurement results. D8-Homocystine, dithiothreitol, and acetonitrile were used as an internal standard, the reducing agent, and the protein precipitating agent, respectively. A triple quadrupole mass spectrometer with an electrospray ionization source was used for monitoring the transitions (m/z 140.0 → 94.0, 136.0 → 90.0) in multiple-reaction-monitoring mode...
March 7, 2017: Analytical and Bioanalytical Chemistry
https://www.readbyqxmd.com/read/28258516/oxidative-stress-in-homocystinuria-due-to-cystathionine-%C3%A3-synthase-deficiency-findings-in-patients-and-in-animal-models
#3
REVIEW
Jéssica Lamberty Faverzani, Tatiane Grazieli Hammerschmidt, Angela Sitta, Marion Deon, Moacir Wajner, Carmen Regla Vargas
Homocystinuria is an inborn error of amino acid metabolism caused by deficiency of cystathionine ß-synthase (CBS) activity, biochemically characterized by homocysteine (Hcy) and methionine (Met) accumulation in biological fluids and high urinary excretion of homocystine. Clinical manifestations include thinning and lengthening of long bones, osteoporosis, dislocation of the ocular lens, thromboembolism, and mental retardation. Although the pathophysiology of this disease is poorly known, the present review summarizes the available experimental findings obtained from patients and animal models indicating that oxidative stress may contribute to the pathogenesis of homocystinuria...
March 3, 2017: Cellular and Molecular Neurobiology
https://www.readbyqxmd.com/read/28241805/rapidly-progressive-psychotic-symptoms-triggered-by-infection-in-a-patient-with-methylenetetrahydrofolate-reductase-deficiency-a-case-report
#4
Shin Iida, Masataka Nakamura, Shinya Asayama, Takenobu Kunieda, Satoshi Kaneko, Hitoshi Osaka, Hirofumi Kusaka
BACKGROUND: Methylenetetrahydrofolate reductase (MTHFR) deficiency is a rare inborn error of metabolism inherited in autosomal recessive pattern and is associated with a wide spectrum of neurological abnormalities. CASE PRESENTATION: We herein describe a 15-year-old boy with MTHFR deficiency who presented with a slowly progressive decline of school performance and a spastic gait. Rapidly deteriorating psychosis and repetitive seizures triggered by a febrile infection prompted neurological investigation...
February 28, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28036059/methionine-and-choline-supply-during-the-periparturient-period-alter-plasma-amino-acid-and-one-carbon-metabolism-profiles-to-various-extents-potential-role-in-hepatic-metabolism-and-antioxidant-status
#5
Zheng Zhou, Mario Vailati-Riboni, Daniel N Luchini, Juan J Loor
The objective of this study was to profile plasma amino acids (AA) and derivatives of their metabolism during the periparturient period in response to supplemental rumen-protected methionine (MET) or rumen-protected choline (CHOL). Forty cows were fed from -21 through 30 days around parturition in a 2 × 2 factorial design a diet containing MET or CHOL. MET supply led to greater circulating methionine and proportion of methionine in the essential AA pool, total AA, and total sulfur-containing compounds. Lysine in total AA also was greater in these cows, indicating a better overall AA profile...
December 29, 2016: Nutrients
https://www.readbyqxmd.com/read/27562997/-thiol-peroxidase-activities-in-rat-blood-plasma-determined-with-hydrogen-peroxide-and-5-5-dithio-bis-2-nitrobenzoic-acid
#6
A V Razygraev, K I Taborskaya, M A Petrosyan, Zh N Tumasova
Earlier it has been shown that extracellular glutathione peroxidase (GPx3) from human plasma is able to use cysteine (Cys-SH) instead of glutathione (GSH) as a thiol substrate. In the present study, the ability of rat plasma to utilize not only GSH, but also Cys-SH and homocysteine (Hcy-SH), in the thiol peroxidase reaction has been confirmed. The molar ratio between thiol and H2O2 in the catalyzed reaction was 2:1. The specific activity increased with fractionation of proteins. At a fixed thiol concentration of 0...
May 2016: Biomedit︠s︡inskai︠a︡ Khimii︠a︡
https://www.readbyqxmd.com/read/26895495/development-of-a-reliable-method-based-on-ultra-performance-liquid-chromatography-coupled-to-tandem-mass-spectrometry-to-measure-thiol-associated-oxidative-stress-in-whole-blood-samples
#7
Javier Escobar, Ángel Sánchez-Illana, Julia Kuligowski, Isabel Torres-Cuevas, Rønnaug Solberg, Håvard T Garberg, Marianne U Huun, Ola D Saugstad, Máximo Vento, Consuelo Cháfer-Pericás
The aminothiols are biological compounds with numerous vital functions. One of the most relevant is their role as antioxidant systems. The reduced to oxidized ratios are extremely useful indicators of oxidative stress and cellular redox status. We have validated an ultra-high performance liquid chromatography coupled to tandem mass spectrometry (UPLC-MS/MS) method to determine the levels of glutathione, cysteine, homocysteine, and their respective oxidized compounds in whole blood samples. Results showed excellent linearity for all the analytes with correlation coefficients between 0...
May 10, 2016: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/25638462/newborn-screening-for-homocystinuria-revealed-a-high-frequency-of-mat-i-iii-deficiency-in-iberian-peninsula
#8
Ana Marcão, María L Couce, Célia Nogueira, Helena Fonseca, Filipa Ferreira, José M Fraga, M Dolores Bóveda, Laura Vilarinho
Homocystinuria due to cystathionine β-synthase deficiency or "classical homocystinuria" is a rare autosomal recessive condition resulting in altered sulfur metabolism with elevated methionine and homocysteine in plasma and homocystine in urine. This condition is characterized by a high clinical heterogeneity, which contributes to late clinical diagnosis, usually only made after irreversible damage has occurred. Treatment is effective if started before clinical symptoms. The analysis of methionine levels by tandem mass spectrometry (MS/MS) allows the newborn screening for homocystinuria, but false-positive results can be frequently obtained and lead to the unwanted identification of methionine adenosyl transferase (MAT I/III) deficiency...
2015: JIMD Reports
https://www.readbyqxmd.com/read/25611566/plasma-amino-acid-profiling-in-major-depressive-disorder-treated-with-selective-serotonin-reuptake-inhibitors
#9
Hye-In Woo, Mi-Ryung Chun, Jeong-Soo Yang, Shinn-Won Lim, Min-Ji Kim, Seon-Woo Kim, Woo-Jae Myung, Doh-Kwan Kim, Soo-Youn Lee
AIMS: Amino acids are important body metabolites and seem to be helpful for understanding pathogenesis and predicting therapeutic response in major depressive disorder (MDD). We performed amino acid profiling to discover potential biomarkers in major depressive patients treated with selective serotonin reuptake inhibitors (SSRIs). METHODS: Amino acid profiling using aTRAQ™ kits for Amino Acid Analysis in Physiological Fluids on a liquid chromatography-tandem mass spectrometry (LC-MS/MS) system was performed on 158 specimens at baseline and at 6 weeks after the initiation of SSRI treatment for 68 patients with MDD and from 22 healthy controls...
May 2015: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/25194423/aortic-stiffness-and-left-ventricular-function-in-patients-with-differentiated-thyroid-cancer
#10
A Gazdag, E V Nagy, A Erdei, M Bodor, E Berta, Z Szabó, Z Jenei
OBJECTIVE: The aim of this study was to investigate aortic stiffness and left ventricular (LV) systolic and diastolic function in patients with differentiated thyroid cancer (DTC) on thyroxine (L-T4) therapy and after L-T4 withdrawal to assess the cardiovascular impact of long-term subclinical hyperthyroidism and short-term overt hypothyroidism. METHODS: Twenty-four patients who had had total thyroidectomy and radioiodine ablation for differentiated thyroid cancer were studied on two occasions: on TSH suppressive L-T4 therapy (sTSH 0...
February 2015: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/24967680/revisiting-synthetic-preparation-of-the-quorum-sensing-substrate-s-d-ribosyl-l-homocysteine-srh
#11
Megan E Bolitho, Brendan J Corcoran, Emily I Showell-Rouse, Keeshia Q Wang
Cleavage of the thioether bond of S-D-ribosyl-L-homocysteine (SRH) by the enzyme S-ribosylhomocysteinase (LuxS) serves as the final biosynthetic step in the generation of the quorum sensing autoinducer AI-2 by bacteria. Herein, a revised chemical synthesis of SRH is presented at convenient scale and purity for in vitro studies of LuxS. Potassium bis(trimethylsilyl)amide (KHMDS) is identified as a judicious base for the formation of the thioether of the target compound from readily-accessible precursors: a thiol nucleophile derived from l-homocystine and a sulfonate-activated d-ribosyl electrophile...
July 23, 2014: Carbohydrate Research
https://www.readbyqxmd.com/read/24456905/disulfide-stress-a-novel-type-of-oxidative-stress-in-acute-pancreatitis
#12
Mari-Luz Moreno, Javier Escobar, Alicia Izquierdo-Álvarez, Anabel Gil, Salvador Pérez, Javier Pereda, Inés Zapico, Máximo Vento, Luis Sabater, Anabel Marina, Antonio Martínez-Ruiz, Juan Sastre
Glutathione oxidation and protein glutathionylation are considered hallmarks of oxidative stress in cells because they reflect thiol redox status in proteins. Our aims were to analyze the redox status of thiols and to identify mixed disulfides and targets of redox signaling in pancreas in experimental acute pancreatitis as a model of acute inflammation associated with glutathione depletion. Glutathione depletion in pancreas in acute pancreatitis is not associated with any increase in oxidized glutathione levels or protein glutathionylation...
May 2014: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/24448992/homocysteine-as-a-predictor-of-early-neurological-deterioration-in-acute-ischemic-stroke
#13
RANDOMIZED CONTROLLED TRIAL
Hyung-Min Kwon, Yong-Seok Lee, Hee-Joon Bae, Dong-Wha Kang
BACKGROUND AND PURPOSE: Hyperhomocysteinemia is a well-known risk factor for vascular disease. However, its action, mechanism, and role in the acute phase of stroke have not been determined. We tried to determine whether an association existed between elevated serum homocysteine levels and early neurological deterioration (END) in patients with acute ischemic stroke. METHODS: We performed a secondary analysis from the Cilostazol in Acute Ischemic Stroke Treatment (CAIST) trial, which was a double-blinded, randomized, multicenter trial, assessing the noninferiority of cilostazol over aspirin within 48 hours of an acute ischemic stroke...
March 2014: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/24302617/determination-of-cysteine-homocysteine-cystine-and-homocystine-in-biological-fluids-by-hplc-using-fluorosurfactant-capped-gold-nanoparticles-as-postcolumn-colorimetric-reagents
#14
Lijuan Zhang, Biqi Lu, Chao Lu, Jin-Ming Lin
We have demonstrated for the first time the suitability of fluorosurfactant-capped spherical gold nanoparticles as HPLC postcolumn colorimetric reagents for the direct assay of cysteine, homocysteine, cystine, and homocystine. The success of this work was based on the use of an on-line tris(2-carboxyethyl)phosphine reduction column for cystine and homocystine. Several parameters affecting the separation efficiency and the postcolumn colorimetric detection were thoroughly investigated. Under the optimized conditions, cysteine, homocysteine, cystine, and homocystine in human urine and plasma samples were determined...
January 2014: Journal of Separation Science
https://www.readbyqxmd.com/read/24263693/dti-correlates-of-cognition-in-conventional-mri-of-normal-appearing-brain-in-patients-with-clinical-features-of-subacute-combined-degeneration-and-biochemically-proven-vitamin-b-12-deficiency
#15
COMPARATIVE STUDY
P K Gupta, R K Gupta, R K Garg, Y Rai, B Roy, C M Pandey, H S Malhotra, P A Narayana
BACKGROUND AND PURPOSE: Vitamin B12 deficiency may cause neural injury that results in cognitive deficits. The main purpose of our study was to evaluate morphometric and microstructural changes in the brain and relate them to cognition in subacute combined degeneration of the spinal cord and patients with biochemically deficient vitamin B12. MATERIALS AND METHODS: Fifty-one patients were recruited and underwent nerve-conduction velocity tests and routine hematologic examinations...
May 2014: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/24206934/dietary-practices-in-pyridoxine-non-responsive-homocystinuria-a-european-survey
#16
S Adam, M F Almeida, E Carbasius Weber, H Champion, H Chan, A Daly, M Dixon, K Dokoupil, D Egli, S Evans, F Eyskens, A Faria, C Ferguson, P Hallam, M Heddrich-Ellerbrok, J Jacobs, C Jankowski, R Lachmann, R Lilje, R Link, S Lowry, K Luyten, A MacDonald, C Maritz, E Martins, U Meyer, E Müller, E Murphy, L V Robertson, J C Rocha, I Saruggia, P Schick, J Stafford, L Stoelen, A Terry, R Thom, T van den Hurk, M van Rijn, A van Teefelen-Heithoff, D Webster, F J White, J Wildgoose, H Zweers
BACKGROUND: Within Europe, the management of pyridoxine (B6) non-responsive homocystinuria (HCU) may vary but there is limited knowledge about treatment practice. AIM: A comparison of dietetic management practices of patients with B6 non-responsive HCU in European centres. METHODS: A cross-sectional audit by questionnaire was completed by 29 inherited metabolic disorder (IMD) centres: (14 UK, 5 Germany, 3 Netherlands, 2 Switzerland, 2 Portugal, 1 France, 1 Norway, 1 Belgium)...
December 2013: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/24103366/cu-ii-disulfide-complexes-display-simultaneous-superoxide-dismutase-and-catalase-like-activities
#17
Margarita E Aliaga, Daniela Andrade-Acuña, Camilo López-Alarcón, Cristián Sandoval-Acuña, Hernán Speisky
Superoxide is a potentially toxic by-product of cellular metabolism. We have addressed here the in vitro ability of complexes formed between copper(II) ions and various biologically-occurring disulfides (RSSR: oxidized glutathione, cystine, homocystine and α-lipoic acid) to react with superoxide. The studied complexes were found to react with superoxide (generated by a xanthine/xanthine oxidase system) at rate constants (kCu(II)-RSSR) close to 10(6)M(-1)s(-1), which are three orders of magnitude lower than that reported for superoxide dismutase (SOD) but comparable to that of several other copper-containing complexes reported as SOD mimetics...
December 2013: Journal of Inorganic Biochemistry
https://www.readbyqxmd.com/read/23973726/neurologically-normal-development-of-a-patient-with-severe-methionine-adenosyltransferase-i-iii-deficiency-after-continuing-dietary-methionine-restriction
#18
Koichi Hirabayashi, Masaaki Shiohara, Kazuhiro Yamada, Akane Sueki, Yuichiro Ide, Koichi Takeuchi, Rokuro Hagimoto, Tatsuya Kinoshita, Akihiko Yabuhara, S Harvey Mudd, Kenichi Koike
BACKGROUND: There is not much information on established standard therapy for patients with severe methionine adenosyltransferase (MAT) I/III deficiency. CASE PRESENTATION: We report a boy with MAT I/III deficiency, in whom plasma methionine and total homocysteine, and urinary homocystine were elevated. Molecular genetic studies showed him to have novel compound heterozygous mutations of the MAT1A gene: c.191T>A (p.M64K) and c.589delC (p.P197LfsX26). A low methionine milk diet was started at 31 days of age, and during continuing dietary methionine restriction plasma methionine levels have been maintained at less than 750 μmol/L...
November 1, 2013: Gene
https://www.readbyqxmd.com/read/23933346/plasma-homocysteine-levels-in-patients-with-multiple-sclerosis-in-the-greek-population
#19
Evangelia Kararizou, George Paraskevas, Nikolaos Triantafyllou, George Koutsis, Maria E Evangelopoulos, Dimitrios Mandellos, Constantinos Sfagos, Elisabeth Kapaki
BACKGROUND: In recent years, there has been increasing interest in the role of plasma homocysteine (Hcy) as a possible risk factor for several diseases of the central nervous system. The aim of this study was to determine the plasma levels of Hcy in a group of multiple sclerosis (MS) patients from a Greek population and the possible correlation with age, disability status, activity or duration of disease, sex, and treatment. METHODS: The MS group that was studied consisted of 46 patients and a total of 42 healthy individuals served as a control group...
November 2013: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/23837610/hydroxysafflor-yellow-a-ameliorates-homocysteine-induced-alzheimer-like-pathologic-dysfunction-and-memory-synaptic-disorder
#20
Ya-Qin Lu, Yu Luo, Zhong-Fang He, Jun Chen, Bo-Ling Yan, Ying Wang, Qin Yu
Homocysteine (Hcy) could induce amyloid β (Aβ) accumulation, synaptic dysfunction, and memory impairment as seen in Alzheimer disease (AD), the most prevalent neurodegenerative disorder, which affects more than 25 million people worldwide. Here we investigated the protective effect of hydroxysafflor yellow A (HSYA) on Hcy-induced Aβ accumulation, synaptic dysfunction, and learning and memory deficits. Rats were randomly divided into four groups: Control group, which received normal saline (NS); Hcy group, which received a daily vena caudalis injection of Hcy (400 μg/kg per day); Hcy+HSYA group, which received the same amount of Hcy plus 6 mg/kg per day HSYA intraperitoneally; and HSYA group, which received 6 mg/kg per day HSYA intraperitoneally for 2 weeks...
December 2013: Rejuvenation Research
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