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Yu-Feng Song, Yan Gao, Christer Hogstrand, Dan-Dan Li, Ya-Xiong Pan, Zhi Luo
Although the role of methionine (Met), as precursor for l-carnitine synthesis, in the regulation of lipid metabolism has been explored. Met seems to have tissue- and species-specific regulatory effect on lipid metabolism, implying that the mechanisms in Met regulation of lipid metabolism is complex and may involve the upstream regulatory pathway of lipid metabolism. The present study was performed to determine the mechanism of apoptosis signaling pathways mediating Met-induced changes of hepatic lipid deposition and metabolism in fish, and compare the differences of the mechanisms between the fish and mammals...
August 9, 2018: Cellular Signalling
Emily R Vasiljevski, Matthew A Summers, David G Little, Aaron Schindeler
Lipid storage myopathies (LSMs) are a heterogeneous group of genetic disorders that present with abnormal lipid storage in multiple body organs, typically muscle. Patients can clinically present with cardiomyopathy, skeletal muscle weakness, myalgia, and extreme fatigue. An early diagnosis is crucial, as some LSMs can be managed by simple nutraceutical supplementation. For example, high dosage l-carnitine is an effective intervention for patients with Primary Carnitine Deficiency (PCD). This review discusses the clinical features and management practices of PCD as well as Neutral Lipid Storage Disease (NLSD) and Multiple Acyl-CoA Dehydrogenase Deficiency (MADD)...
August 9, 2018: Progress in Lipid Research
M S Andrianova, E V Kuznetsov, V P Grudtsov, A E Kuznetsov
A CMOS-compatible ISFET with a Ta2 O5 sensitive surface was developed. The structure was optimized for achieving high sensitivity using a subthreshold operation mode and by reducing the influence of the capacitances on the value of subthreshold swing. The developed ISFET was used as a basis for a biosensor for L-carnitine detection. To this end, carnitine acetyltransferase was immobilized on the ISFET sensitive surface. The immobilized enzyme was active (0.082 U/g model plate ). The complete microsystem, consisting of a packaged chip, an immobilized enzyme and a microfluidic channel, detected L-carnitine at a range of 0...
July 24, 2018: Biosensors & Bioelectronics
Kaihui Zhang, Hongying Li, Xiaoying Li, Yi Liu, Zhongtao Gai
OBJECTIVE: To analyze the clinical features and genetic basis of a female neonate with muscle weakness, abnormal brain magnetic resonance imaging and elevated blood lactate. METHODS: The patient was subjected to clinical and laboratory examination. Next generation sequencing was carried out for the patient and her relatives. RESULTS: The proband was diagnosed as small for gestational age, with clinical features including muscle weakness, abnormal brain magnetic resonance imaging, increased blood lactate, and acidosis...
August 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Masatsugu Ohara, Koji Ogawa, Goki Suda, Megumi Kimura, Osamu Maehara, Tomoe Shimazaki, Kazuharu Suzuki, Akihisa Nakamura, Machiko Umemura, Takaaki Izumi, Naoki Kawagishi, Masato Nakai, Takuya Sho, Mitsuteru Natsuizaka, Kenichi Morikawa, Shunsuke Ohnishi, Naoya Sakamoto
Liver cirrhosis (LC) is a major cause of secondary sarcopenia. Sarcopenia makes the prognosis worse; thus, novel therapeutic options for sarcopenia in patients with LC are urgently required as they are currently limited. In this retrospective study, 158 patients with LC were screened, and 35 of those patients who were treated with L-carnitine for more than 6 months and for whom skeletal muscle mass changes could be evaluated by computer tomography were enrolled. Of the 158 patients, 79 patients who did not receive L-carnitine supplementation served as controls...
August 2018: Hepatology communications
Lais Pancotto, Ricieri Mocelin, Matheus Marcon, Ana P Herrmann, Angelo Piato
Studies have suggested that oxidative stress may contribute to the pathogenesis of mental disorders. In this context, molecules with antioxidant activity may be promising agents in the treatment of these deleterious conditions. Acetyl-L-carnitine (ALC) is a multi-target molecule that modulates the uptake of acetyl-CoA into the mitochondria during fatty acid oxidation, acetylcholine production, protein, and membrane phospholipid synthesis, capable of promoting neurogenesis in case of neuronal death. Moreover, neurochemical effects of ALC include modulation of brain energy and synaptic transmission of multiple neurotransmitters, including expression of type 2 metabotropic glutamate (mGlu2) receptors...
2018: PeerJ
Navid Dadashpour Davachi, Roozbeh Fallahi, Essa Dirandeh, Xinyu Liu, Pawel M Bartlewski
Developmental potential of cryopreserved in vitro matured oocytes is very low in nearly all mammalian species studied to date. Despite relatively high cleavage rates, the vitrified/warmed metaphase II oocytes have a decreased rate of blastocyst formation, which can be attributed to the elevated cytoplasmic lipid content and lipid droplet fragmentation. Secretory products of ampulla oviductal epithelial cells (AECs) at the periovulatory stage of the ovarian cycle enhance the viability of in vitro matured oocytes...
July 29, 2018: Theriogenology
Florian Jacques, Sonia Rippa, Yolande Perrin
L-carnitine is present in all living kingdoms where it acts in diverse physiological processes. It is involved in lipid metabolism in animals and yeasts, notably as an essential cofactor of fatty acid intracellular trafficking. Its physiological significance is poorly understood in plants, but L-carnitine may be linked to fatty acid metabolism among other roles. Indeed, carnitine transferases activities and acylcarnitines are measured in plant tissues. Current knowledge of fatty acid trafficking in plants rules out acylcarnitines as intermediates of the peroxisomal and mitochondrial fatty acid metabolism, unlike in animals and yeasts...
September 2018: Plant Science: An International Journal of Experimental Plant Biology
Aliki Anyfantaki, Christiana Kyvelidou, Margarita Tsagkaraki, George Filippidis, Mattheos Fraidakis, Alexandros Zafiropoulos, Irene Athanassakis
Implantation failure is a major problem in human assisted reproduction, which persists regardless the optimization of endometrial receptivity and selection of genetically and morphologically healthy embryos. Since embryo-endometrium interaction depends on cell junctional, cell adhesion and cell-substratum adhesion molecules, the present study inquired whether in vitro growing murine embryos display similar to the in vivo growing embryos patterns of adhesion molecules. To this extend aVb3 expression and distribution in zygotes and 2-cell stage embryos were studied...
August 3, 2018: Reproductive Biology
Min Kyoung Kim, Jae Kyun Park, Soo Kyung Paek, Ji Won Kim, In Pyung Kwak, Hee Jun Lee, Sang Woo Lyu, Woo Sik Lee
AIM: Antioxidants have been studied to be effective in improving embryo qualities from in vitro fertilization. L-carnitine (LC) has been known to reduce reactive oxygen species and enhance adenosine triphosphate production, which contribute to the development of a high-quality embryo. This is the first study to include both mouse and human subjects and aimed to evaluate whether LC supplementation in culture media has any beneficial effect on the development of the embryos, as well as its clinical outcomes...
August 1, 2018: Journal of Obstetrics and Gynaecology Research
Carla Nasca, Benedetta Bigio, Francis S Lee, Sarah P Young, Marin M Kautz, Ashly Albright, James Beasley, David S Millington, Aleksander A Mathé, James H Kocsis, James W Murrough, Bruce S McEwen, Natalie Rasgon
The lack of biomarkers to identify target populations greatly limits the promise of precision medicine for major depressive disorder (MDD), a primary cause of ill health and disability. The endogenously produced molecule acetyl-l-carnitine (LAC) is critical for hippocampal function and several behavioral domains. In rodents with depressive-like traits, LAC levels are markedly decreased and signal abnormal hippocampal glutamatergic function and dendritic plasticity. LAC supplementation induces rapid and lasting antidepressant-like effects via epigenetic mechanisms of histone acetylation...
July 30, 2018: Proceedings of the National Academy of Sciences of the United States of America
S Dinicola, A Fuso, A Cucina, M Santiago-Reyes, R Verna, V Unfer, G Monastra, M Bizzarri
OBJECTIVE: Cancer patients frequently experience Chemotherapy-Induced Peripheral Neuropathy (CIPN), as a typical side effect related to time of administration and dose of anticancer agents. Yet, CIPN pathophysiology is poorly understood, and there is a lack of well-tolerated pharmacological remedies helpful to prevent or treat it. Therefore, new safe and effective compounds are highly warranted, namely if based on an adequate understanding of the pathogenic mechanisms. MATERIAL AND METHODS: Herein we reviewed and discussed scientific data related to the beneficial role of some non-conventional treatments able to counteract CIPN, focusing our attention on alpha-lipoic acid (ALA) and L-acetyl-carnitine (LAC), two natural products that have been demonstrated to be promising preventive drugs...
July 2018: European Review for Medical and Pharmacological Sciences
Agata Chmurzynska, Agnieszka Seremak-Mrozikiewicz, Anna M Malinowska, Agata Różycka, Anna Radziejewska, Artur Szwengiel, Grażyna Kurzawińska, Magdalena Barlik, Paweł P Jagodziński, Krzysztof Drews
Maternal metabolism during gestation may depend on nutrient intake but also on polymorphism of genes encoding enzymes involved in metabolism of different nutrients. Data on choline or carnitine metabolism in pregnant women are scarce. We hypothesized that (1) choline intake in Polish pregnant women is inadequate and (2) choline and carnitine metabolism would differ by genotype and nutritional status of pregnant women. One hundred three healthy Polish women aged 18 to 44 years in the third trimester of pregnancy were enrolled in the study...
August 2018: Nutrition Research
Hui Chen, Yik Lung Chan, Claire Linnane, Yilin Mao, Ayad G Anwer, Arjun Sapkota, Tiara F Annissa, George Herok, Bryce Vissel, Brian G Oliver, Sonia Saad, Catherine A Gorrie
There is a need for pharmaceutical agents that can reduce neuronal loss and improve functional deficits following traumatic brain injury (TBI). Previous research suggests that oxidative stress and mitochondrial dysfunction play a major role in neuronal damage after TBI. Therefore, this study aimed to investigate two drugs known to have antioxidant effects, L-carnitine and exendin-4, in rats with moderate contusive TBI. L-carnitine (1.5 mM in drinking water) or exendin-4 (15 µg/kg/day, ip) were given immediately after the injury for 2 weeks...
July 25, 2018: Scientific Reports
Michelle du Plessis, Jaco Franken, Florian F Bauer
L-Carnitine is a key metabolite in the energy metabolism of eukaryotic cells, functioning as a shuttling molecule for activated acyl-residues between cellular compartments. In higher eukaryotes this function is essential, and defects in carnitine metabolism has severe effects on fatty acid and carbon metabolism. Carnitine supplementation has been associated with an array of mostly beneficial impacts in higher eukaryotic cells, including stress protection and regulation of redox metabolism in diseased cells...
2018: Frontiers in Microbiology
Roya Modarresi, Alireza Aminsharifi, Farzaneh Foroughinia
PURPOSE: To evaluate the efficacy of therapy with spirulina supplement on semen parameters in patients withidiopathic male infertility. MATERIALS AND METHODS: A total of 40 men with idiopathic infertility were randomly assigned into two groups. Group A received 2 g spirulina supplement as well as conventional regimen for the treatment of infertility selected by their physician (220 mg/day zinc sulfate, 500mg/day L-carnitine, and 50 mg/day clomiphene) during 12 weeks of the study, while group B received placebo plus conventional therapy during the study period...
July 23, 2018: Urology Journal
Millad Ramazani, Durdi Qujeq, Zoleika Moazezi
OBJECTIVES: This study is essentially a correlative study that examines the potential of reduced levels of L-carnitine (LC) when combined with the pathophysiology of type 2 diabetes. The aim of the study was to assess the levels of LC, total antioxidant capacity (TAOC), fasting blood sugar (FBS), triglycerides and cholesterol in people with newly diagnosed and long-standing type 2 diabetes and in healthy controls. METHODS: The study was done in 90 adult subjects, including 30 with newly diagnosed diabetes, 30 with long-standing type 2 diabetes and 30 healthy controls...
March 29, 2018: Canadian Journal of Diabetes
H D Vallance, A Koochin, J Branov, A Rosen-Heath, T Bosdet, Z Wang, S L Hazen, G Horvath
Oral supplementation with l-carnitine is a common therapeutic modality for mitochondrial disorders despite limited evidence of efficacy. Recently, a number of studies have demonstrated that a gut microbiota-dependent metabolite of l-carnitine, trimethylamine oxide (TMAO), is an independent and dose-dependent risk factor for cardiovascular disease (CVD). Given the limited data demonstrating efficacy with oral l-carnitine therapy and the newly raised questions of potential harm, we assessed plasma TMAO levels in patients with mitochondrial disease with and without oral l-carnitine supplementation...
June 2018: Molecular Genetics and Metabolism Reports
Kenji Watanabe, Kenji Yamada, Koji Sameshima, Seiji Yamaguchi
Introduction: Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an autosomal recessive mitochondrial fatty acid oxidation disorder and presents as hypoketotic hypoglycemia or rhabdomyolysis during childhood. l-Carnitine supplementation for patients with VLCAD deficiency is controversial. Herein, we describe two siblings with VLCAD deficiency who experienced more frequent episodes of rhabdomyolysis after l-carnitine supplementation. Case presentation: Case 1 involved a 6-year-old boy who was diagnosed with VLCAD deficiency after repeated episodes of hypoketotic hypoglycemia at 3 years of age...
June 2018: Molecular Genetics and Metabolism Reports
Lea Katalinic, Branimir Krtalic, Bojan Jelakovic, Nikolina Basic-Jukic
BACKGROUND/AIMS: There is a growing body of evidence that the long-term hemodialysis (HD) treatment leads to disturbances of carnitine homeostasis but the results of L-carnitine supplementation in HD patients have been conflicting. In the present prospective study, we investigated the effectiveness of intravenous L-carnitine in mitigating dialysis-related protein-energy wasting (PEW) based on pre-treatment albumin levels. METHODS: Fifty patients (46% male, mean age 63±18...
July 17, 2018: Kidney & Blood Pressure Research
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