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Carnitine

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https://www.readbyqxmd.com/read/29148268/development-and-validation-of-a-uhplc-hrms-ms-quantification-method-for-hypoglycin-a-and-mcpa-carnitine-in-horse-serum-in-cases-of-atypical-myopathy
#1
Wiebke Rudolph, Daniela Remane, Dirk K Wissenbach, Carmen Klein, Dirk Barnewitz, Frank T Peters
Atypical myopathy (AM) is a fatal disease in horses presumably caused by hypoglycin A (HGA) from ingested maple seeds and its active metabolite methylenecyclopropyl-acetic-acid (MCPA). The aim of this study was the development and validation of a rapid and simple assay for HGA and MCPA-carnitine in horse serum and its application to authentic samples. Identification and quantification were carried out by high resolution LC-MS/MS with fullscan/data-dependent MS(2) . Chromatographic separation was performed by isocratic elution on a HILIC column (100 x 2...
November 17, 2017: Drug Testing and Analysis
https://www.readbyqxmd.com/read/29147969/effect-of-dietary-phospholipid-levels-on-growth-lipid-metabolism-and-antioxidative-status-of-juvenile-hybrid-snakehead-channa-argus%C3%A3-channa-maculata
#2
Shi-Mei Lin, Fa-Jian Li, Bundit Yuangsoi, Sompong Doolgindachbaporn
The study was conducted to evaluate the effect of dietary phospholipids (PLs) on growth, lipid metabolism, and antioxidative status of hybrid snakehead (Channa argus × Channa maculata). Five isonitrogenous and isolipidic diets with graded levels of PLs (8.5, 19.3, 30.7, 41.5, and 50.8 g kg(-1)) were fed to triplicate groups of juveniles (initial body weight 12.6 ± 0.23 g) for 8 weeks. Results showed that dietary PL supplementation significantly improved growth of juveniles. The final body weight (FBW) and specific growth rate (SGR) significantly increased with dietary PLs increasing from 8...
November 16, 2017: Fish Physiology and Biochemistry
https://www.readbyqxmd.com/read/29139118/di-2-ethylhexyl-phthalate-dehp-exposure-disturbs-lipid-metabolism-in-juvenile-yellow-catfish-tachysurus-fulvidraco
#3
F X Meng, M Li, M Z Song, L X Yuan, Y F Gong, Y X Qian, G Shi, R X Wang
This study was conducted to determine the mechanism by which di-2-ethylhexyl phthalate (DEHP) exposure influences lipid metabolism of juvenile yellow catfish Tachysurus fulvidraco. Fish were exposed to three DEHP concentrations (0, 0·1 and 0·5 mg l(-1) DEHP) for 8 weeks. Fatty acid synthase (FAS) activity significantly decreased with increasing DEHP concentrations, the highest value was in the Tween control group, whereas the lowest activities of carnitine palmitoyltransferase (CPT) and lipoprotein lipase (LPL) were in this group...
November 15, 2017: Journal of Fish Biology
https://www.readbyqxmd.com/read/29139026/structural-elucidation-of-novel-biomarkers-of-known-metabolic-disorders-based-on-multistage-fragmentation-mass-spectra
#4
Jan Václavík, Karlien L M Coene, Ivo Vrobel, Lukáš Najdekr, David Friedecký, Radana Karlíková, Lucie Mádrová, Aleksanteri Petsalo, Udo F H Engelke, Annemiek van Wegberg, Leo A J Kluijtmans, Tomáš Adam, Ron A Wevers
Specific diagnostic markers are the key to effective diagnosis and treatment of inborn errors of metabolism (IEM). Untargeted metabolomics allows for the identification of potential novel diagnostic biomarkers. Current separation techniques coupled to high-resolution mass spectrometry provide a powerful tool for structural elucidation of unknown compounds in complex biological matrices. This is a proof-of-concept study testing this methodology to determine the molecular structure of as yet uncharacterized m/z signals that were significantly increased in plasma samples from patients with phenylketonuria and 3-hydroxy-3-methylglutaryl-CoA lyase deficiency...
November 14, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29138817/reduced-triglyceride-accumulation-due-to-overactivation-of-farnesoid-x-receptor-signaling-contributes-to-impaired-liver-regeneration-following-50-hepatectomy-in-extra%C3%A2-cholestatic-liver-tissue
#5
Wen-Jun Jia, Shi-Quan Sun, Luo-Shun Huang, Qiao-Li Tang, Yu-Dong Qiu, Liang Mao
The aim of the present study was to investigate the role of triglyceride metabolism in the effect of obstructive cholestasis on liver regeneration following 50% partial hepatectomy (PH). Obstructive cholestatic rat models were achieved via ligation of the common bile duct (BDL). Following comparisons between hepatic pathological alterations with patients with perihilar cholangiocarcinoma, rats in the 7 day post‑BDL group were selected as the BDL model for subsequent experiments. Liver weight restoration, proliferating cell nuclear antigen labeling index, cytokine and growth factor expression levels, and hepatic triglyceride content were evaluated to analyze liver regeneration post‑PH within BDL and control group rats...
November 10, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29137181/structure-and-functional-analysis-of-promoters-from-two-liver-isoforms-of-cpt-i-in-grass-carp-ctenopharyngodon-idella
#6
Yi-Huan Xu, Zhi Luo, Kun Wu, Yao-Fang Fan, Wen-Jing You, Li-Han Zhang
Carnitine palmitoyltransferase I (CPT I) is a key enzyme involved in the regulation of lipid metabolism and fatty acid β-oxidation. To understand the transcriptional mechanism of CPT Iα1b and CPT Iα2a genes, we cloned the 2695-bp and 2631-bp regions of CPT Iα1b and CPT Iα2a promoters of grass carp (Ctenopharyngodon idella), respectively, and explored the structure and functional characteristics of these promoters. CPT Iα1b had two transcription start sites (TSSs), while CPT Iα2a had only one TSS. DNase I foot printing showed that the CPT Iα1b promoter was AT-rich and TATA-less, and mediated basal transcription through an initiator (INR)-independent mechanism...
November 13, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29137068/carnitine-acylcarnitine-translocase-deficiency-with-c-199-10-t-g-and-novel-c-1a-g-mutation-two-case-reports-and-brief-literature-review
#7
Hui-Ming Yan, Hao Hu, Aisha Ahmed, Bing-Bing Feng, Jing Liu, Zheng-Jun Jia, Hua Wang
RATIONALE: Carnitine-acylcarnitine translocate deficiency (CACTD) is a rare and life-threatening, autosomal recessive disorder of fatty acid β-oxidation characterized by hypoketotic hypoglycemia, hyperammonemia, cardiomyopathy, liver dysfunction, and muscle weakness; culminating in early death. To date, CACTD cases screened from the Chinese mainland population, especially patient with compound heterozygote with c.199-10T>G and a novel c.1A>G mutation in the SLC25A20 gene has never been described...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29136144/antioxidants-improve-ivf-outcome-and-subsequent-embryo-development-in-the-mouse
#8
T Truong, D K Gardner
STUDY QUESTION: What is the effect of a combination of three antioxidants (Acetyl-L-Carnitine, N-Acetyl-L-Cysteine and α-Lipoic Acid), present in IVF medium during mouse oocyte and sperm collection, on fertilization and subsequent IVF embryo development? SUMMARY ANSWER: A combination of antioxidants resulted in faster developmental times from the 2-cell stage through to expanded blastocyst stage, accompanied by a significant increase in blastocyst cell number and a reduction of intracellular hydrogen peroxide (H2O2) levels...
November 10, 2017: Human Reproduction
https://www.readbyqxmd.com/read/29132536/effects-of-acute-hyperinsulinemia-on-skeletal-muscle-mitochondrial-function-reactive-oxygen-species-production-and-metabolism-in-premenopausal-women
#9
Jonathan L Warren, Sule Bulur, Fernando Ovalle, Samuel T Windham, Barbara A Gower, Gordon Fisher
BACKGROUND: Acute metabolic demands that promote excessive and/or prolonged reactive oxygen species production may stimulate changes in mitochondrial oxidative capacity. PURPOSE: To assess changes in skeletal muscle H2O2 production, mitochondrial function, and expression of genes at the mRNA and protein levels regulating energy metabolism and mitochondrial dynamics following a hyperinsulinemic-euglycemic clamp in a cohort of 11 healthy premenopausal women. METHODS: Skeletal muscle biopsies of the vastus lateralis were taken at baseline and immediately following the conclusion of a hyperinsulinemic-euglycemic clamp...
December 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/29132460/-genetic-diagnosis-of-10-neonates-with-primary-carnitine-deficiency
#10
Jian-Qiang Tan, Da-Yu Chen, Zhe-Tao Li, Ti-Zhen Yan, Ji-Wei Huang, Ren Cai
OBJECTIVE: To study the gene mutation profile of primary carnitine deficiency (PCD) in neonates, and to provide a theoretical basis for early diagnosis and treatment, genetic counseling, and prenatal diagnosis of PCD. METHODS: Acylcarnitine profile analysis was performed by tandem mass spectrometry using 34 167 dry blood spots on filter paper. The SLC22A5 gene was sequenced and analyzed in neonates with free carnitine (C0) levels lower than 10 μmol/L as well as their parents...
November 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29131297/gut-bacteria-derived-molecules-as-mediators-and-markers-in-cardiovascular-diseases-the-role-of-the-gut-blood-barrier
#11
Marcin Ufnal, Artur Nowiński
Accumulating evidence suggests that gut bacteria-derived compounds enter the bloodstream and affect the circulatory system functions. For example, hydrogen sulfide, a product of gut sulfate-reducing bacteria, was found to exert significant hemodynamic effects. Furthermore, trimethylamine N-oxide, a gut bacteria derivative metabolite of L-carnitine and choline, has recently emerged as a new marker of increased cardiovascular risk. To enter the circulation, gut bacteria metabolites need to pass the gut-blood barrier...
November 13, 2017: Kardiologia Polska
https://www.readbyqxmd.com/read/29129455/plasma-palmitoyl-carnitine-ac16-0-is-a-marker-of-increased-postprandial-nonesterified-incomplete-fatty-acid-oxidation-rate-in-individuals-with-type-2-diabetes
#12
Fatima-Zahra Bouchouirab, Mélanie Fortin, Christophe Noll, Jean Dubé, André C Carpentier
OBJECTIVES: Enhanced mitochondrial fatty acid utilization is known to increase radical oxidative stress and induce insulin resistance. An increased level of plasma acylcarnitine (AC) has been proposed to indicate mitochondrial energy substrate overload, a possible mechanism leading to insulin resistance. The aim of our study was to determine fasting and postprandial plasma acetyl-carnitine (AC2:0), palmitoyl-carnitine (AC16:0), oleoyl-carnitine (AC18:1) and linoleoyl-carnitine (AC18:2) levels and their relationships with plasma nonesterified fatty acid appearance and oxidation rates and insulin sensitivity in participants with type 2 diabetes and normoglycemic offspring of 2 parents with type 2 diabetes (FH+) compared to healthy participants without family histories of type 2 diabetes (FH-)...
November 9, 2017: Canadian Journal of Diabetes
https://www.readbyqxmd.com/read/29127404/investigation-of-novel-metabolites-potentially-involved-in-the-pathogenesis-of-coronary-heart-disease-using-a-uhplc-qtof-ms-based-metabolomics-approach
#13
Yiping Li, Dong Zhang, Yuan He, Changzhe Chen, Chenxi Song, Yanyan Zhao, Yinxiao Bai, Yang Wang, Jielin Pu, Jingzhou Chen, Yuejin Yang, Kefei Dou
Coronary heart disease (CHD) is associated with complex metabolic disorders, but its molecular aetiology remains unclear. Using a novel nontargeted metabolomics approach, we explored the global metabolic perturbation profile for CHD. Blood samples from 150 patients with severe obstructive CHD and 150 angiographically normal controls were collected. Metabolic fingerprinting was performed by ultra-high performance liquid chromatography coupled to quadruple time-of-flight mass spectrometry (UHPLC-QTOF/MS) technique...
November 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29124685/mitochondrial-trifunctional-protein-deficiency-severe-cardiomyopathy-and-cardiac-transplantation
#14
C Bursle, R Weintraub, C Ward, R Justo, J Cardinal, D Coman
We describe mitochondrial trifunctional protein deficiency (MTPD) in two male siblings who presented with severe cardiomyopathy in infancy. The first sibling presented in severe cardiac failure at 6 months of age and succumbed soon after. The second sibling came to attention after newborn screening identified a possible fatty acid oxidation defect. Dietary therapy and carnitine supplementation commenced in the neonatal period. Despite this the second child required cardiac transplantation at 3 years of age after a sudden and rapid decline in cardiac function...
November 10, 2017: JIMD Reports
https://www.readbyqxmd.com/read/29116185/targeted-metabolomics-reveals-abnormal-hepatic-energy-metabolism-by-depletion-of-%C3%AE-carotene-oxygenase-2-in-mice
#15
Lei Wu, Xin Guo, Yi Lyu, Stephen L Clarke, Edralin A Lucas, Brenda J Smith, Deana Hildebrand, Weiqun Wang, Denis M Medeiros, Xinchun Shen, Dingbo Lin
β-carotene oxygenase 2 (BCO2) is a carotenoid cleavage enzyme located in the inner mitochondrial membrane. Ablation of BCO2 impairs mitochondrial function leading to oxidative stress. Herein, we performed a targeted metabolomics study using ultrahigh performance liquid chromatography-tandem mass spectroscopy and gas chromatography-mass spectroscopy to discriminate global metabolites profiles in liver samples from six-week-old male BCO2 systemic knockout (KO), heterozygous (Het), and wild type (WT) mice fed a chow diet...
November 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29115530/curcumin-suppresses-notch%C3%A2-1-signaling-improvements-in-fatty-liver-and-insulin-resistance-in-rats
#16
Neng-Jiang Zhao, Ming-Juan Liao, Jing-Jing Wu, Ke-Xin Chu
Curcumin is a well‑known phenolic substance and has many pharmacological effects associated with metabolism. However, the exact molecular mechanisms underlying this process have yet to be determined. The Notch pathway is a signal transduction pathway involved in energy metabolism. The present study aimed to investigate the effects of curcumin administration on glucose‑lipid metabolism in rats subjected to a high fat diet, and investigate changes in Notch‑1 signaling. Sprague‑Dawley rats (n=40) were randomly divided into four groups (10 rats/group): Control diet group, high fat diet group, high fat diet plus curcumin low dose group and high fat diet plus curcumin high dose group...
November 6, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29112178/efficacy-and-effectiveness-of-carnitine-supplementation-for-cancer-related-fatigue-a-systematic-literature-review-and-meta-analysis
#17
REVIEW
Wolfgang Marx, Laisa Teleni, Rachelle S Opie, Jaimon Kelly, Skye Marshall, Catherine Itsiopoulos, Elizabeth Isenring
BACKGROUND: Carnitine deficiency has been implicated as a potential pathway for cancer-related fatigue that could be treated with carnitine supplementation. The aim of this systematic literature review and meta-analysis was to evaluate the literature regarding the use of supplemental carnitine as a treatment for cancer-related fatigue. METHODS: Using the PRISMA guidelines, an electronic search of the Cochrane Library, MEDLINE, Embase, CINAHL and reference lists was conducted...
November 7, 2017: Nutrients
https://www.readbyqxmd.com/read/29110324/cnta-oxygenase-substrate-profile-comparison-and-oxygen-dependency-of-tma-production-in-providencia-rettgeri
#18
Gints Kalnins, Eduards Sevostjanovs, Dace Hartmane, Solveiga Grinberga, Kaspars Tars
CntA oxygenase is a Rieske 2S-2Fe cluster-containing protein that has been previously described as able to produce trimethylamine (TMA) from carnitine, gamma-butyrobetaine, glycine betaine, and in one case, choline. TMA found in humans is exclusively of bacterial origin, and its metabolite, trimethylamine oxide (TMAO), has been associated with atherosclerosis and heart and renal failure. We isolated four different Rieske oxygenases and determined that there are no significant differences in their substrate panels...
November 7, 2017: Journal of Basic Microbiology
https://www.readbyqxmd.com/read/29109780/metabolomic-profiling-of-extracellular-vesicles-and-alternative-normalization-methods-reveal-enriched-metabolites-and-strategies-to-study-prostate-cancer-related-changes
#19
Maija Puhka, Maarit Takatalo, Maria-Elisa Nordberg, Sami Valkonen, Jatin Nandania, Maria Aatonen, Marjo Yliperttula, Saara Laitinen, Vidya Velagapudi, Tuomas Mirtti, Olli Kallioniemi, Antti Rannikko, Pia R-M Siljander, Taija Maria Af Hällström
Body fluids are a rich source of extracellular vesicles (EVs), which carry cargo derived from the secreting cells. So far, biomarkers for pathological conditions have been mainly searched from their protein, (mi)RNA, DNA and lipid cargo. Here, we explored the small molecule metabolites from urinary and platelet EVs relative to their matched source samples. As a proof-of-concept study of intra-EV metabolites, we compared alternative normalization methods to profile urinary EVs from prostate cancer patients before and after prostatectomy and from healthy controls...
2017: Theranostics
https://www.readbyqxmd.com/read/29103113/testicular-toxicity-and-sperm-quality-following-copper-exposure-in-wistar-albino-rats-ameliorative-potentials-of-l-carnitine
#20
Maurya Khushboo, Meesala Krishna Murthy, Maibam Sunita Devi, Sanasam Sanjeev, Kalibulla Syed Ibrahim, Nachimuthu Senthil Kumar, Vikas Kumar Roy, Guruswami Gurusubramanian
Copper is a persistent toxic and bio-accumulative heavy metal of global concern. Continuous exposure of copper compounds of different origin is the most common form of copper poisoning and in turn adversely altering testis morphology and function and affecting sperm quality. L-carnitine has a vital role in the spermatogenesis, physiology of sperm, sperm production and quality. This study was designed to examine whether the detrimental effects of long-term copper consumption on sperm quality and testis function of Wistar albino rat could be prevented by L-carnitine therapy...
November 4, 2017: Environmental Science and Pollution Research International
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