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https://www.readbyqxmd.com/read/28914566/a-novel-etfdh-mutation-in-an-adult-patient-with-late-onset-riboflavin-responsive-multiple-acyl-coa-dehydrogenase-deficiency
#1
Min Chen, Jing Peng, Wei Wei, Wang, Hongliang Xu, Hongbo Liu
We report a novel mutation in the electron transfer flavoprotein dehydrogenase (ETFDH) gene in an adult patient with late-onset riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency (MADD) characterized by muscle weakness and hypoglycemia as main clinical presentation. At the age of 34 years, the patient experienced progressive muscle weakness and short of breath. Blood creatine kinase level was significantly higher than normal. The blood glucose was significantly lower than normal. The muscle biopsy revealed lipid storage myopathy...
September 15, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28911246/dual-targeting-of-l-carnitine-conjugated-nanoparticles-to-octn2-and-atb-0-to-deliver-chemotherapeutic-agents-for-colon-cancer-therapy
#2
Longfa Kou, Qing Yao, Sathish Sivaprakasam, Qiuhua Luo, Yinghua Sun, Qiang Fu, Zhonggui He, Jin Sun, Vadivel Ganapathy
l-Carnitine, obligatory for oxidation of fatty acids, is transported into cells by the Na(+)-coupled transporter OCTN2 and the Na(+)/Cl(-)-coupled transporter ATB(0,+). Here we investigated the potential of L-carnitine-conjugated poly(lactic-co-glycolic acid) (PLGA) nanoparticles (LC-PLGA NPs) to deliver chemotherapeutic drugs into cancer cells by targeting the nanoparticles to both OCTN2 and ATB(0,+). The cellular uptake of LC-PLGA NPs in the breast cancer cell line MCF7 and the colon cancer cell line Caco-2 was increased compared to unmodified nanoparticles, but decreased in the absence of co-transporting ions (Na(+) and/or Cl(-)) or in the presence of competitive substrates for the two transporters...
November 2017: Drug Delivery
https://www.readbyqxmd.com/read/28877455/global-analysis-of-plasma-lipids-identifies-liver-derived-acylcarnitines-as-a-fuel-source-for-brown-fat-thermogenesis
#3
Judith Simcox, Gisela Geoghegan, John Alan Maschek, Claire L Bensard, Marzia Pasquali, Ren Miao, Sanghoon Lee, Lei Jiang, Ian Huck, Erin E Kershaw, Anthony J Donato, Udayan Apte, Nicola Longo, Jared Rutter, Renate Schreiber, Rudolf Zechner, James Cox, Claudio J Villanueva
Cold-induced thermogenesis is an energy-demanding process that protects endotherms against a reduction in ambient temperature. Using non-targeted liquid chromatography-mass spectrometry-based lipidomics, we identified elevated levels of plasma acylcarnitines in response to the cold. We found that the liver undergoes a metabolic switch to provide fuel for brown fat thermogenesis by producing acylcarnitines. Cold stimulates white adipocytes to release free fatty acids that activate the nuclear receptor HNF4α, which is required for acylcarnitine production in the liver and adaptive thermogenesis...
September 5, 2017: Cell Metabolism
https://www.readbyqxmd.com/read/28872093/-research-progress-of-trimethylamine-n-oxide-in-the-pathogenesis-of-atherosclerosis
#4
Huahua He, Xinfu Lian, Zhiqun Tang
Trimethylamine-N-oxide (TMAO), metabolites of the intestinal microflora, is a newly discovered risk factor for cardiovascular disease. The intestinal flora converted choline and L-carnitine into trimethylamine in the food. Trimethylamine is oxidized to TMAO in liver enzymes. Lowering TMA can stimulate macrophages to reverse cholesterol transport and inhibit atherogenesis. TMAO poietin-monooxygenase 3 (FMO3) is a tool for cholesterol metabolism and reverse cholesterol transpor, lowering FMO3 can slow the gallbladder's secretion of bile, delay intestinal absorption of cholesterol, and limit the synthesis of oxidized cholesterol and cholesterol esters...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28870581/pharmacological-treatments-for-fatigue-in-patients-with-multiple-sclerosis-a-systematic-review-and-meta-analysis
#5
Ting-Ting Yang, Li Wang, Xiao-Yang Deng, Gang Yu
BACKGROUND: Multiple sclerosis (MS) is a chronic immune-mediated inflammatory disease. Fatigue is the most common symptom of MS patients, affecting >80% subjects. Medical treatment is an important method for managing fatigue. Currently, although many drugs have been tested in treatment of MS fatigue, the efficacy of these drugs remain largely unclear. METHODS: We researched available literatures in PubMed, Embase, Medline, Google Scholar, Cochrane Library (August 31, 2016)...
September 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28866892/plasma-l-carnitine-levels-in-terminally-ill-cancer-patients-receiving-only-palliative-care
#6
Akiyuki Sakamoto, Yoshiko Tsukahara, Daisuke Gomi, Toshirou Fukushima, Takashi Kobayashi, Hirohide Matsushita, Nodoka Sekiguchi, Keiko Mamiya, Tomonobu Koizumi
BACKGROUND: Several studies indicated that plasma L-carnitine (LC) levels are significantly decreased during chemotherapy or chemoradiation and that LC supplementation can improve the fatigue score in some cancer patients. However, the LC levels in end-stage cancer patients treated only with palliative care remained unclear. The present study was performed to examine the plasma LC levels of terminally ill and hospitalized patients. METHODS: Twenty-one terminally ill cancer patients in our hospital, with expected survival of several months, were enrolled in the present study...
August 2017: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/28862226/serum-metabolomics-in-oral-leukoplakia-and-oral-squamous-cell-carcinoma
#7
Gokul Sridharan, Pratibha Ramani, Sangeeta Patankar
CONTEXT: Metabolomics is a core discipline of system biology focusing on the study of low molecular weight compounds in biological system. Analysis of human metabolome, which is composed of diverse group of metabolites, can aid in diagnosis and prognosis of oral squamous cell carcinoma (OSCC). AIM: The aim of the present study is to analyze and identify serum metabolites in oral leukoplakia and OSCC as a potential diagnostic biomarker and a predictor for malignant transformation of oral leukoplakia...
July 2017: Journal of Cancer Research and Therapeutics
https://www.readbyqxmd.com/read/28855839/ameliorative-effects-of-l-carnitine-on-rats-raised-on-a-diet-supplemented-with-lead-acetate
#8
El-Said El-Sherbini, Gehad El-Sayed, Rehab El Shotory, Nervana Gheith, Mohamed Abou-Alsoud, Steve Mustapha Harakeh, Gamal I Karrouf
Lead intoxication has been a major health hazard in humans. It affects people at all ages. Its toxicity is associated with various organs of the body and affects different metabolic pathways. Based on histological data, l-carnitine reduced the severity of tissue damage produced as a result of exposure of rats to lead acetate. The main objective of this study was to evaluate the underlying mechanism of protection offered by l-carnitine against lead acetate intoxication using male Sprague-Dawley rats. Forty male Sprague-Dawley rats were randomly divided into four groups with ten rats in each...
September 2017: Saudi Journal of Biological Sciences
https://www.readbyqxmd.com/read/28845935/-using-l-and-acetyl-l-carnintines-in-combination-with-clomiphene-citrate-and-antioxidant-complex-for-treating-idiopathic-male-infertility-a-prospective-randomized-trial
#9
V A Bozhedomov, N A Lipatova, G E Bozhedomova, I M Rokhlikov, E V Shcherbakova, R A Komarina
The most common cause of male infertility is idiopathic oligo-, and or astheno-, and /or teratozoospermia. In such cases, anti-estrogens, antioxidants (vitamins and trace elements) or carnitines are used, but the evidence on their effectiveness is inconsistent; there are currently no published studies exploring their concurrent use. AIM: To investigate the efficacy and safety of the L- and acetyl-L-carnitine complex, vitamins A, E, C, selenium, zinc and other antioxidants ("SpermActin" + "More than vitamins") in combination with clomiphene citrate (CC) in managing male idiopathic infertility in the form of oligo, and/or astheno-, and/or teratozoospermia...
July 2017: Urologii︠a︡
https://www.readbyqxmd.com/read/28845931/-adjuvant-antioxidant-therapy-in-varicocele-infertility
#10
S I Gamidov, R I Ovchinnikov, A Yu Popova, A Yu Avakyan, G T Sukhikh
INTRODUCTION: Infertility affects an estimated 15% of couples globally. Male factor infertility accounts for about a half of infertility cases. About 40% of infertile men have varicocele. The aim of this study was to investigate the efficacy and safety of a complex of acetyl-L-carnitine, L-carnitine fumarate and alpha-lipoic acid (SpermActin-forte) (SA) for adjuvant antioxidant therapy after microsurgical varicocelectomy (MVE) in men with varicocele and assess its impact on the level of DNA fragmentation in sperm cells...
June 2017: Urologii︠a︡
https://www.readbyqxmd.com/read/28838027/n-butyl-4-hydroxy-1-8-naphthalimide-a-new-matrix-for-small-molecule-analysis-by-matrix-assisted-laser-desorption-ionization-mass-spectrometry
#11
Xina Cheng, Xueting Ye, Dan Liu, Nan Zhao, Huiyuan Gao, Ping Wang, Guangbo Ge, Xiaozhe Zhang
RATIONALE: The matrix plays an essential role in defining detection limits and ionization yields of analytes in matrix-assisted laser desorption/ionization mass spectrometry (MALDI-MS) analysis. Small molecule MALDI-MS analyses commonly suffer from the high background interference generated from matrices. Moreover, the inhomogeneous crystallization of some matrices, such as 2,5-dihydroxybenzoic acid (DHB), is to the detriment of the quality or repeatability of detection. We have found that N-butyl-4-hydroxy-1,8-naphthalimide (BHN) can provide improved performance as a matrix for small molecule analysis...
August 24, 2017: Rapid Communications in Mass Spectrometry: RCM
https://www.readbyqxmd.com/read/28837408/intestinal-surfactant-permeation-enhancers-and-their-interaction-with-enterocyte-cell-membranes-in-a-mucosal-explant-system
#12
E Michael Danielsen, Gert H Hansen
Intestinal permeation enhancers (PEs) are agents aimed to improve oral delivery of therapeutic drugs with poor bioavailability. The main permeability barrier for oral delivery is the intestinal epithelium, and PEs act to increase the paracellular and/or transcellular passage of drugs. Transcellular passage can be achieved by cell membrane permeabilization and/or by endocytic uptake and subsequent transcytosis. One broad class of PEs is surfactants which act by inserting into the cell membrane, thereby perturbing its integrity, but little is known about how the dynamics of the membrane are affected...
July 3, 2017: Tissue Barriers
https://www.readbyqxmd.com/read/28834388/dry-eye-disease-and-oxidative-stress
#13
REVIEW
Sophia Seen, Louis Tong
Dry eye, an age-related condition, is a multifactorial disease of the tears and ocular surface that results in symptoms of discomfort, visual disturbance and tear film instability. Environmental factors are also often implicated in dry eye including exposure to pollutants, ultraviolet (UV) radiation and ozone as well as the chronic use of preserved eyedrops such as in the treatment of glaucoma. These factors increase oxidative stress and ocular surface inflammation. Here, we reviewed the cellular, animal and clinical studies that point to the role of oxidative stress in dry eye disease...
August 21, 2017: Acta Ophthalmologica
https://www.readbyqxmd.com/read/28833236/clinical-trial-of-l-carnitine-and-valproic-acid-in-spinal-muscular-atrophy-type-i
#14
Kristin J Krosschell, John T Kissel, Elise L Townsend, Sarah D Simeone, Ren Zhe Zhang, Sandra P Reyna, O Crawford Thomas, Mary K Schroth, Gyula Acsadi, Priya S Kishnani, Jürgen-Christoph von Kleist-Retzow, Barbara Hero, Guy D'Anjou, Edward C Smith, Bakri Elsheikh, Louise R Simard, Thomas W Prior, Charles B Scott, Bernard LaSalle, Ai Sakonju, Brunhilde Wirth, Kathryn J Swoboda
INTRODUCTION: To explore safety and therapeutic potential of L-carnitine and valproic acid (VPA) in infants with spinal muscular atrophy (SMA). METHODS: Open-label phase 2 multicenter trial of L-carnitine and VPA in infants with SMA type I with retrospective comparison to an untreated, matched cohort. Primary outcomes were safety and adverse events (AEs); secondary outcomes were survival, time to death/> 16 hours/day ventilator support, motor outcomes and maximum ulnar compound motor action potential amplitude...
August 18, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28832573/effects-of-l-carnitine-supplementation-on-biomarkers-of-oxidative-stress-antioxidant-capacity-and-lipid-profile-in-patients-with-pemphigus-vulgaris-a-randomized-double-blind-placebo-controlled-trial
#15
H Mohammadi, M Djalali, M Daneshpazhooh, N M Honarvar, C Chams-Davatchi, F Sepandar, Z Fakhri, E Yaghubi, M Zarei, M H Javanbakht
BACKGROUND/OBJECTIVES: Pemphigus vulgaris (PV), as an autoimmune disease including mucosa and the skin, is associated with several complications and comorbidities. The present study planned to determine the effect of L-carnitine (LC) supplementation on biomarkers of oxidative stress (OS), antioxidant capacity and lipid profile in PV patients.Subjects/MethodsFifty two control and patients with PV, participated in the current randomized, double-blind, placebo-controlled clinical trial. The patients were allocated randomly to receive 2 g per day LC tartrate subdivided into two equal doses of 1 g before breakfast and dinner (n=26) or placebo (n=26) for 8 weeks...
August 23, 2017: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28828020/nephroprotective-effects-of-l-carnitine-against-contrast-induced-nephropathy-in-patients-undergoing-percutaneous-coronary-intervention-a-randomized-open-labeled-clinical-trial
#16
Mohammad Mohammadi, Azita Hajhossein Talasaz, Mohammad Alidoosti, Hamid Reza Pour Hosseini, Kheirollah Gholami, Arash Jalali, Hamid Aryannejad
Background: L-carnitine may prevent the incidence of AKI by its antioxidant effects and improving circulation in ischemic conditions. The goal of this trial was to evaluate the impact of L-carnitine on contrast-induced nephropathy in patients undergoing elective PCI. Methods: Totally, the patients were randomly allocated to 2 groups. The treatment group received 1 g of L-carnitine orally 3 times a day, 24 hours before the procedure (3 g before PCI) and 2 g after PCI, whereas the control group did not receive L-carnitine...
April 2017: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/28822835/neuroprotective-effects-of-acetyl-l-carnitine-on-lipopolysaccharide-induced-neuroinflammation-in-mice-involvement-of-brain-derived-neurotrophic-factor
#17
Filiz Kazak, Gul Fatma Yarim
Neuroinflammation is the inflammation of nervous tissue that can lead to neurodegeneration. Brain-derived neurotrophic factor (BDNF) is a neurotrophin which affects growth, function and survival of neurons, enhances the stabilization of synapses, regulates synaptic function and branching of dendrites and axons. Brain-derived neurotrophic factor is believed to be involved in the pathophysiology of central nervous system (CNS) diseases associated with neuroinflamation. The aim of this study was to investigate new protective and therapeutic effect of acetyl-l-carnitine (ALCAR) in neuroinflammation...
August 16, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28807823/dietary-supplementation-with-acetyl-l-carnitine-counteracts-age-related-alterations-of-mitochondrial-biogenesis-dynamics-and-antioxidant-defenses-in-brain-of-old-rats
#18
Luigi Nicassio, Flavio Fracasso, Giuseppe Sirago, Clara Musicco, Anna Picca, Emanuele Marzetti, Riccardo Calvani, Palmiro Cantatore, Maria Nicola Gadaleta, Vito Pesce
We previously reported the ability of dietary supplementation with acetyl-l-carnitine (ALCAR) to prevent age-related decreases of mitochondrial biogenesis in skeletal muscle and liver of old rats. Here, we investigate the effects of ALCAR supplementation in cerebral hemispheres and cerebellum of old rats by analyzing several parameters linked to mitochondrial biogenesis, mitochondrial dynamics and antioxidant defenses. We measured the level of the coactivators PGC-1α and PGC-1β and of the factors regulating mitochondrial biogenesis, finding an age-related decrease of PGC-1β, whereas PGC-1α level was unvaried...
August 12, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28807226/liver-metabolomics-study-reveals-protective-function-of-phyllanthus-urinaria-against-ccl4-induced-liver-injury
#19
Qing Guo, Qian-Qian Zhang, Jia-Qing Chen, Wei Zhang, Hong-Cong Qiu, Zun-Jian Zhang, Bu-Ming Liu, Feng-Guo Xu
Phyllanthus Urinaria L. (PUL) is a traditional Chinese medicine used to treat hepatic and renal disorders. However, the mechanism of its hepatoprotective action is not fully understood. In the present study, blood biochemical indexes and liver histopathological changes were used to estimate the extent of hepatic injury. GC/MS and LC/MS-based untargeted metabolomics were used in combination to characterize the potential biomarkers associated with the protective activity of PUL against CCl4-induced liver injury in rats...
July 2017: Chinese Journal of Natural Medicines
https://www.readbyqxmd.com/read/28802113/l-carnitine-enhances-oocyte-maturation-and-improves-in%C3%A2-vitro-development-of-embryos-in-dromedary-camels-camelus-dromedaries
#20
Mohamed Fathi, K H El-Shahat
The aim of the present study was to investigate the effect of L-carnitine (LC) addition during either IVM or IVC on the developmental potential of camel oocytes. In Experiment 1; camel oocytes were matured in the absence (control) or presence of different concentrations of LC (0.25 mg, 0.5 mg, 0.75 mg and 1 mg/ml) for 30 h followed by in vitro fertilization and culture up to blastocyst stage. Our results demonstrated that oocytes treated with 0.5 mg/ml LC showed higher (P < 0.05) rates of maturation (74...
August 4, 2017: Theriogenology
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