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JOURNAL ARTICLE
Tsugunori Maruyama, Sho Matsui, Ryosuke Kobayashi, Takuro Horii, Yasuo Oguri, Satoshi Tsuzuki, Takahiro Horie, Koh Ono, Izuho Hatada, Tsutomu Sasaki
Most studies on fat appetite have focused on long-chain triglycerides (LCTs) due to their obesogenic properties. Medium-chain triglycerides (MCTs), conversely, exhibit anti-obesogenic effects; however, the regulation of MCTs intake remains elusive. Here, we demonstrate that mice can distinguish between MCTs and LCTs, and the specific appetite for MCTs is governed by hepatic β-oxidation. We generated liver-specific medium-chain acyl-CoA dehydrogenase (MCAD)-deficient (MCADL-/- ) mice and analyzed their preference for MCTs and LCTs solutions using glyceryl trioctanoate (C8-TG), glyceryl tridecanoate (C10-TG), corn oil, and lard oil in two-bottle choice tests conducted over 8 days...
April 10, 2024: American Journal of Physiology. Endocrinology and Metabolism
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JOURNAL ARTICLE
Giulia Pagano, Lydia Sastre, Annabel Blasi, Salvatore Brugaletta, Judit Mestres, Julia Martinez-Ocon, Jose T Ortiz-Pérez, Clara Viñals, Susanna Prat-Gonzàlez, Eva Rivas, Rosario J Perea, Sergio Rodriguez-Tajes, África Muxí, Emilio Ortega, Ada Doltra, Pablo Ruiz, Bàrbara Vidal, Graciela Martínez-Palli, Jordi Colmenero, Gonzalo Crespo
BACKGROUND: The optimal cardiovascular assessment of liver transplant (LT) candidates is unclear. We aimed to evaluate the performance of CT-based coronary tests (coronary artery calcium score [CACS] and coronary CT angiography [CCTA]) and a modification of the CAD-LT score (mCAD-LT, excluding family history of CAD) to diagnose significant coronary artery disease (CAD) before LT and predict the incidence of post-LT cardiovascular events (CVE). METHODS: We retrospectively analysed a single-centre cohort of LT candidates who underwent non-invasive tests; invasive coronary angiography (ICA) was performed depending on the results of non-invasive tests...
April 9, 2024: Liver International: Official Journal of the International Association for the Study of the Liver
#3
Recurrent familial case of early childhood sudden death: Complex post mortem genetic investigations.
Lila Krebs-Drouot, Audrey Schalk, Elise Schaefer, Christine Keyser, Angela Gonzalez, Nadège Calmels, Marie-Thérèse Abi Wardé, Laetitia Oertel, C Écile Acquaviva, Jean-Louis Mandel, Audrey Farrugia
INTRODUCTION: Sudden Unexplained Death in Childhood (SUDC) needs to be fully assessed considering its impact on the family, parents and siblings. Inborn Errors of Metabolism (IEM) such as Medium-Chain Acyl-CoA Dehydrogenase Deficiency (MCADD) should be taken into consideration when SUDC occurres. Our aim is to present a family with two successive SUDC and to discuss the post-mortem genetics investigations revealing an IEM implication. CASES REPORT: A complete autopsy with genetic testing was performed when the proband, a 4-year-old girl, died...
March 2, 2024: Forensic Science International. Genetics
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JOURNAL ARTICLE
Huseyin Bilgin, Ayse Ergul Bozaci
AIM: It was aimed to identify markers that would indicate which cases presenting with rhabdomyolysis are more likely to be associated with inherited metabolic diseases. METHODS: We analyzed 327 children who applied to our Hospital Pediatric Nutrition and Metabolic Diseases Clinic with rhabdomyolysis. The diagnosis of rhabdomyolysis was made by measuring the serum creatinine kinase level in cases presenting with muscle pain, weakness and dark urine. RESULTS: Metabolic disease was detected in 29 (16/13, M/F) patients from 26 different families...
June 2024: Molecular Genetics and Metabolism Reports
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JOURNAL ARTICLE
Shulin Wang, Xuan Zhang, Yanhong Hou, Yuliang Zhang, Jiamin Chen, Shuhan Gao, Huiying Duan, Shaoju Gu, Shanshan Yu, Yi Cai
The Sirtuins family, formally known as the Silent Information Regulator Factors, constitutes a highly conserved group of histone deacetylases. Recent studies have illuminated SIRT6's role in doxorubicin (DOX)-induced oxidative stress and apoptosis within myocardial cells. Nevertheless, the extent of SIRT6's impact on DOX-triggered myocardial cell aging and damage remains uncertain, with the associated mechanisms yet to be fully understood. In our research, we examined the influence of SIRT6 on DOX-induced cardiomyocyte senescence using β-galactosidase and γ-H2AX staining...
February 21, 2024: Chemico-biological Interactions
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JOURNAL ARTICLE
Lina Zhang, Shuguang Ren, Yang Sang, Yueyang Hu, Cong Wang, Xinrui Wang, Yuntao Li
Breast cancer (BC) is among the top three most prevalent cancers across the world, especially in women, and its pathogenesis is still unknown. Fatty acid β-oxidation is highly associated with breast cancer. Serpin family E member 1 (SERPINE1)-induced down-regulation of fatty acid β-oxidation can facilitate BC cell proliferation, invasion, and metastasis. In this paper, the difference of miR-30d-5p expressions in both cancerous tissues and para-carcinoma tissues was first detected. Next, the expressions of SERPINE1, long-chain acyl-CoA dehydrogenase (LCAD) and medium-chain acyl-CoA dehydrogenase (MCAD) in the aforementioned tissues were analyzed...
February 22, 2024: Aging
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JOURNAL ARTICLE
Emilie Everard, Hilde Laeremans, François Boemer, Sandrine Marie, Marie-Françoise Vincent, Joseph P Dewulf, François-Guillaume Debray, Corinne De Laet, Marie-Cécile Nassogne
Fatty acid oxidation (FAO) disorders are autosomal recessive genetic disorders affecting either the transport or the oxidation of fatty acids. Acute symptoms arise during prolonged fasting, intercurrent infections, or intense physical activity. Metabolic crises are characterized by alteration of consciousness, hypoglycemic coma, hepatomegaly, cardiomegaly, arrhythmias, rhabdomyolysis, and can lead to death. In this retrospective and multicentric study, the data of 54 patients with FAO disorders were collected...
February 7, 2024: European Journal of Paediatric Neurology: EJPN
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JOURNAL ARTICLE
Tiantian Liu, Xu Chen, Qianbin Sun, Junjun Li, Qiyan Wang, Peng Wei, Wei Wang, Chun Li, Yong Wang
INTRODUCTION: Valerenic acid (VA) is a unique and biologically active component in Valeriana officinalis L., which has been reported to have a regulatory effect on the cardiovascular system. However, its therapeutic effects on pathological myocardial hypertrophy (PMH) and the underlying mechanisms are undefined. OBJECTIVES: Our study aims to elucidate how VA improves PMH, and preliminarily discuss its mechanism. METHODS: The efficacy of VA on PMH was confirmed by in vivo and in vitro experiments and the underlying mechanism was investigated by molecular dynamics (MD) simulations and specific siRNA interference...
February 17, 2024: Journal of Advanced Research
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JOURNAL ARTICLE
Meng Wang, Lingchen Wang, Liang Zhou, Yizeng Xu, Chen Wang
BACKGROUND: Shen Shuai Ⅱ Recipe (SSR) is clinically used to treat chronic kidney diseases (CKDs) with remarkable efficacy and safety. In earlier research, we found the anti-inflammatory, antioxidant, and mitochondrial protective properties of SSR in hypoxic kidney injury model, which is closely related to its renal protection. Further work is needed to understand the underlying molecular mechanisms. PURPOSE: Further investigation of the mechanisms of action of SSR against renal interstitial fibrosis (RIF) building on previous research leads...
February 12, 2024: Phytomedicine
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JOURNAL ARTICLE
Maryem Ismaili, Samira Krimissa, Mustapha Namous, Kamal Abdelrahman, Abdelghani Boudhar, Mohamed Edahbi, Youssef Lebrini, Abdelaziz Htitiou, Soufiane Maimouni, Tarik Benabdelouhab
To address the increasing global demand for food, it is crucial to implement sustainable agricultural practices, which include effective soil management techniques for enhancing productivity and environmental conditions. In this regard, a study was conducted to assess the efficacy of utilizing phenological metrics derived from satellite data in order to map and identify suitable agricultural soil within a semi-arid region. Two distinct methodologies were compared: one based on physicochemical soil parameters and the other utilizing the phenological response of vegetation through the application of the Normalized Difference Vegetation Index (NDVI) Modis-time series...
January 30, 2024: Heliyon
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JOURNAL ARTICLE
Cleisson Fábio Andrioli Peralta, Karina Jorge Rodrigues da Costa, Ana Clara Peneluppi Horak, Samara Pinheiro do Carmo Gomes, Elton Sousa Santos, Letícia Galvão Barbante, Renato Hideo Nakagawa Santos
OBJECTIVES: To identify predictors of outcomes in severe twin oligo-polyhydramnios sequence (TOPS) with or without twin anemia-polycythemia sequence (TAPS) and/or selective fetal growth restriction (SFGR) treated by laser ablation of placental vessels (LAPV). METHODS: Analysis of cases treated from 2011 to 2022. Variables evaluated Prenatal predictors: stages of TOPS, presence of TAPS and/or SFGR; pre-LAPV fetal ultrasound parameters; peri-LAPV variables. Perinatal predictors: GA at birth; birthweight; Apgar scores; transfontanellar ultrasonography (TFUS)...
January 19, 2024: Prenatal Diagnosis
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JOURNAL ARTICLE
Ryan Iverson, Monica Taljaard, Michael T Geraghty, Michael Pugliese, Kylie Tingley, Doug Coyle, Jonathan B Kronick, Kumanan Wilson, Valerie Austin, Catherine Brunel-Guitton, Daniela Buhas, Nancy J Butcher, Alicia K J Chan, Sarah Dyack, Sharan Goobie, Cheryl R Greenberg, Shailly Jain-Ghai, Michal Inbar-Feigenberg, Natalya Karp, Mariya Kozenko, Erica Langley, Matthew Lines, Julian Little, Jennifer MacKenzie, Bruno Maranda, Saadet Mercimek-Andrews, Aizeddin Mhanni, John J Mitchell, Laura Nagy, Martin Offringa, Amy Pender, Murray Potter, Chitra Prasad, Suzanne Ratko, Ramona Salvarinova, Andreas Schulze, Komudi Siriwardena, Neal Sondheimer, Rebecca Sparkes, Sylvia Stockler-Ipsiroglu, Kendra Tapscott, Yannis Trakadis, Lesley Turner, Clara Van Karnebeek, Anthony Vandersteen, Jagdeep S Walia, Brenda J Wilson, Andrea C Yu, Beth K Potter, Pranesh Chakraborty
BACKGROUND: Generating rigorous evidence to inform care for rare diseases requires reliable, sustainable, and longitudinal measurement of priority outcomes. Having developed a core outcome set for pediatric medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, we aimed to assess the feasibility of prospective measurement of these core outcomes during routine metabolic clinic visits. METHODS: We used existing cohort data abstracted from charts of 124 children diagnosed with MCAD deficiency who participated in a Canadian study which collected data from birth to a maximum of 11 years of age to investigate the frequency of clinic visits and quality of metabolic chart data for selected outcomes...
January 13, 2024: BMC Pediatrics
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JOURNAL ARTICLE
Kewei Wang, Rong Zhang, Nadja Lehwald, Guo-Zhong Tao, Bowen Liu, Bo Liu, Yangseok Koh, Karl G Sylvester
BACKGROUND AND AIMS: Wnt/β-catenin signaling plays an important role in regulating hepatic metabolism. This study is to explore the molecular mechanisms underlying the potential crosstalk between Wnt/β-catenin and mTOR signaling in hepatic steatosis. METHODS: Transgenic mice (overexpress Wnt1 in hepatocytes, Wnt+) mice and wild-type littermates were given high fat diet (HFD) for 12 weeks to induce hepatic steatosis. Mouse hepatocytes cells (AML12) and those transfected to cause constitutive β-catenin stabilization (S33Y) were treated with oleic acid for lipid accumulation...
2023: Frontiers in Endocrinology
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JOURNAL ARTICLE
Su-Wen Chen, Guo-Liang Yin, Chao-Yuan Song, De-Cheng Meng, Wen-Fei Yu, Xin Zhang, Ya-Nan Feng, Peng-Peng Liang, Feng-Xia Zhang
This study aims to observe the effects of diosgenin on the expression of mammalian target of rapamycin(mTOR), sterol regulatory element-binding protein-1c(SREBP-1c), heat shock protein 60(HSP60), medium-chain acyl-CoA dehydrogenase(MCAD), and short-chain acyl-CoA dehydrogenase(SCAD) in the liver tissue of the rat model of non-alcoholic fatty liver disease(NAFLD) and explore the mechanism of diosgenin in alleviating NAFLD. Forty male SD rats were randomized into five groups: a control group, a model group, low-(150 mg·kg~(-1)·d~(-1)) and high-dose(300 mg·kg~(-1)·d~(-1)) diosgenin groups, and a simvastatin(4 mg·kg~(-1)·d~(-1)) group...
October 2023: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
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JOURNAL ARTICLE
Xiangyi-Yi Zeng, Arshad Javid, Gang Tian, Ke-Ying Zhang, Shi-Ping Bai, Xue-Mei Ding, Jian-Ping Wang, Li Lv, Yue Xuan, Shan-Shan Li, Qiu-Feng Zeng
Heat stress (HS) is a major environmental threat that affects duck production in subtropical and tropical regions, especially in summer. This study aimed to evaluate the physiological and metabolic responses of Pekin ducks to chronic HS conditions via liquid chromatography-mass spectrometry (LC-MS) using a paired-fed (PF) experimental design. On the basis of equivalent feed intake (HS vs. PF), HS significantly reduced growth performance and the percentage of leg and breast muscles, however, markedly increased the percentage of abdominal fat and breast skin fat...
December 16, 2023: Science of the Total Environment
#16
JOURNAL ARTICLE
Homa Faraji, Azadeh Ebrahim-Habibi
Acyl-CoA dehydrogenase deficiency (ACAD) is an inherited and potentially fatal disorder with variable clinical symptoms. The relationship between pathogenicity and deleterious point mutations is investigated here in ACAD structures of short (SCAD) and medium-chain (MCAD) types. Structures and dynamic features of native and mutant forms of enzymes models were compared. A total of 2.88 µs molecular dynamics simulations were performed at four different temperatures. Total energy, RMSD, protein ligand interactions and affinity, RMSF measures, secondary structure changes, and important interactions were studied...
December 16, 2023: Journal of Biological Physics
#17
JOURNAL ARTICLE
Shohei Matsufuji, Yoshihiko Kitajima, Kazuki Higure, Naoya Kimura, Sachiko Maeda, Kohei Yamada, Kotaro Ito, Tomokazu Tanaka, Keita Kai, Hirokazu Noshiro
BACKGROUND: A hypoxic environment often persists within solid tumors, including hepatocellular carcinoma (HCC). Hypoxia-inducible factor-1α (HIF-1α) can accelerate cancer malignancy by inducing hypoxia-dependent expression of various genes. Tumor hypoxia can also induce metabolic reprogramming of fatty acid (FA) metabolism, through which HIF-1α plays an essential role in diminishing fatty acid β-oxidation (FAO) in hypoxic cancer cells. METHODS: We aimed to investigate potential new drug therapy options for targeting hypoxic cancer cells within HCC tumors, specifically through combining HIF-1α inhibition with palmitic acid (PA) + L-carnitine (LC) treatment to effectively induce apoptosis in hypoxic HCC cells...
December 8, 2023: Cancer & Metabolism
#18
JOURNAL ARTICLE
Kate L Weeks, Helen Kiriazis, Glenn D Wadley, Emma I Masterman, Nicola M Sergienko, Antonia J A Raaijmakers, Adam J Trewin, Claudia A Harmawan, Gunes S Yildiz, Yingying Liu, Brian G Drew, Paul Gregorevic, Lea M D Delbridge, Julie R McMullen, Bianca C Bernardo
Diabetic cardiomyopathy describes heart disease in patients with diabetes who have no other cardiac conditions but have a higher risk of developing heart failure. Specific therapies to treat the diabetic heart are limited. A key mechanism involved in the progression of diabetic cardiomyopathy is dysregulation of cardiac energy metabolism. The aim of this study was to determine if increasing the expression of medium-chain acyl-coenzyme A dehydrogenase (MCAD; encoded by Acadm), a key regulator of fatty acid oxidation, could improve the function of the diabetic heart...
November 21, 2023: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
#19
JOURNAL ARTICLE
Fukai Wang, Xiaowei Ma, Jing Ye, Chenzhi Shi, Yun Chen, Zhicai Yu, Tianming Li, Donglei Yang, Min Li, Pengfei Wang
Detection of viral infections (e.g., SARS-CoV-2) with high precision is critical to disease control and treatment. There is an urgent need to develop point-of-care detection methods to complement the gold standard laboratory-based PCR assay with comparable sensitivity and specificity. Herein, we developed a method termed mCAD to achieve ultraspecific point-of-care detection of SARS-CoV-2 RNA while maintaining high sensitivity by programming multiplex rolling circle amplification and toehold-mediated strand displacement reactions...
November 16, 2023: Analytical Chemistry
#20
JOURNAL ARTICLE
Maria Kipele, Michael Buchfelder, R Verena Taudte, Andreas Stadlbauer, Thomas Kinfe, Yavor Bozhkov
Objective : Metabolomics has growing importance in the research of inflammatory processes. Chronic subdural hematoma (cSDH) is considered to be, at least in part, of inflammatory nature, but no metabolic analyses yet exist. Therefore, a mass spectrometry untargeted metabolic analysis was performed on hematoma samples from patients with cSDH. Methods : A prospective analytical cross-sectional study on the efficacy of subperiosteal drains in cSDH was performed. Newly diagnosed patients had the option of granting permission for the collection of a hematoma sample upon its removal...
October 30, 2023: Diagnostics
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