keyword
https://read.qxmd.com/read/38483976/a-fatty-acid-anabolic-pathway-in-specialized-cells-sustains-a-remote-signal-that-controls-egg-activation-in-drosophila
#1
JOURNAL ARTICLE
Mickael Poidevin, Nicolas Mazuras, Gwénaëlle Bontonou, Pierre Delamotte, Béatrice Denis, Maëlle Devilliers, Perla Akiki, Delphine Petit, Laura de Luca, Priscilla Soulie, Cynthia Gillet, Claude Wicker-Thomas, Jacques Montagne
Egg activation, representing the critical oocyte-to-embryo transition, provokes meiosis completion, modification of the vitelline membrane to prevent polyspermy, and translation of maternally provided mRNAs. This transition is triggered by a calcium signal induced by spermatozoon fertilization in most animal species, but not in insects. In Drosophila melanogaster, mature oocytes remain arrested at metaphase-I of meiosis and the calcium-dependent activation occurs while the oocyte moves through the genital tract...
March 14, 2024: PLoS Genetics
https://read.qxmd.com/read/38448864/a-chromosome-level-genome-assembly-of-pseudoroegneria-libanotica-reveals-a-key-kcs-gene-involves-in-the-cuticular-wax-elongation-for-drought-resistance
#2
JOURNAL ARTICLE
Xingguang Zhai, Dandan Wu, Chen Chen, Xunzhe Yang, Shaobo Cheng, Lina Sha, Shuhan Deng, Yiran Cheng, Xing Fan, Houyang Kang, Yi Wang, Dengcai Liu, Yonghong Zhou, Haiqin Zhang
BACKGROUND: The genus Pseudoroegneria (Nevski) Löve (Triticeae, Poaceae), whose genome symbol was designed as "St", accounts for more than 60% of perennial Triticeae species. The diploid species Psudoroegneria libanotica (2n = 14) contains the most ancient St genome, exhibited strong drought resistance, and was morphologically covered by cuticular wax on the aerial part. Therefore, the St-genome sequencing data could provide fundamental information for studies of genome evolution and reveal its mechanisms of cuticular wax and drought resistance...
March 6, 2024: BMC Genomics
https://read.qxmd.com/read/38422897/the-3-hydroxyacyl-coa-dehydratase-1-2-form-complex-with-trans-2-enoyl-coa-reductase-involved-in-substrates-transfer-in-very-long-chain-fatty-acid-elongation
#3
JOURNAL ARTICLE
Youli Zhou, Rui Lv, Richard D Ye, Ruobing Ren, Leiye Yu
Very long-chain fatty acids (VLCFAs) are fatty acids with a carbon chain length greater than 18 carbons (>C18) and exhibit various functions, such as in skin barrier formation, liver homeostasis, myelin maintenance, spermatogenesis, retinal function, and anti-inflammation. VLCFAs are absorbed by dietary or elongated from endogenous hexadecanoyl acids (C16). Similar to long-chain fatty acid synthesis, VLCFAs elongation begins with acyl-CoA and malonyl-CoA as sources, and the length of the acyl chain is extended by two carbon units in each cycle...
February 2, 2024: Biochemical and Biophysical Research Communications
https://read.qxmd.com/read/38387522/toxicological-evaluation-of-a-fish-oil-concentrate-containing-very-long-chain-fatty-acids
#4
JOURNAL ARTICLE
Derek Tobin, Harald Svensen, Devanand Shanmugasundaram, Bente Ruyter, Iren Stoknes, Michael Dornish
Very long chain fatty acids (VLCFA) have a chain length ≥24 carbons. Fish contain low levels of these fatty acids. A commercial oil called EPAX® Evolve 05 with an up-concentration of VLCFAs of approximately 10 times, has been developed as a dietary supplement by Epax Norway AS. A series of toxicological studies were performed using mice and rats to determine the safety and toxicity of repeat dosing with a gavage administered VLCFA formulation. The results suggest transient lipid accumulation in kidneys and liver...
February 20, 2024: Food and Chemical Toxicology
https://read.qxmd.com/read/38381602/msc-derived-small-extracellular-vesicles-exert-cardioprotective-effect-through-reducing-vlcfas-and-apoptosis-in-human-cardiac-organoid-iri-model
#5
JOURNAL ARTICLE
Boon Min Poh, Lee Chuen Liew, Yan Ni Annie Soh, Ruenn Chai Lai, Sai Kiang Lim, Ying Swan Ho, Boon Seng Soh
Cardiovascular diseases (CVDs) are the leading cause of death worldwide, accounting for 31% of all deaths globally. Myocardial ischemia-reperfusion injury (IRI), a common complication of CVDs, is a major cause of mortality and morbidity. Studies have shown efficacious use of mesenchymal stem cells-derived extracellular vesicles (MSCs-EVs) to mitigate IRI in animals, but few research has been done on human-related models. In this study, human embryonic stem cell-derived chambered cardiac organoid (CCOs) was used as a model system to study the effects of MSC-derived small extracellular vesicles (sEVs) on myocardial IRI...
February 21, 2024: Stem Cells
https://read.qxmd.com/read/38370996/the-clinical-spectrum-of-adrenoleukodystrophy-at-a-portuguese-tertiary-hospital-case-series-and-review-of-literature
#6
Catarina Menezes, Ana Losa, Sara Mosca, Ana de Carvalho Vaz, Catarina M Figueiredo, Cristina Garrido, Teresa Borges, Joana Borges Correia
Adrenoleukodystrophy, a rare genetic disease associated with the X chromosome (X-ALD - X-linked adrenoleukodystrophy), predominantly affects males and stems from mutations in the ABCD1 gene, responsible for transporting very long chain fatty acids (VLCFA) into peroxisomes. It leads to adrenal insufficiency (AI) and axonal demyelination. In males, the phenotype varies from isolated adrenocortical insufficiency and progressive myelopathy to cerebral adrenoleukodystrophy (CALD). The aim of this case series is to characterize patients with different clinical presentations of X-ALD with follow-up at a tertiary Portuguese hospital...
January 2024: Curēus
https://read.qxmd.com/read/38320654/plasma-c24-0-and-c26-0-lysophosphatidylcholines-are-reliable-biomarkers-for-the-diagnosis-of-peroxisomal-%C3%AE-oxidation-disorders
#7
JOURNAL ARTICLE
Blai Morales-Romero, José Manuel González de Aledo-Castillo, Cristina Fernández Sierra, Carmen Martínez Carreira, Carles Zaragoza Bonet, Rosa Fernández Bonifacio, Maria Antònia Caro Miró, Ana Argudo-Ramírez, Rosa María López Galera, Judit García-Villoria
The gold-standard diagnostic test for peroxisomal disorders (PDs) is plasma concentration analysis of very long-chain fatty acids (VLCFAs). However, this method's time-consuming nature and limitations in cases which present normal VLCFA levels necessitates alternative approaches. The analysis of C26:0-lysophosphatydylcholine (C26:0-LPC) in dried blood spot samples by tandem-mass spectrometry (MS/MS) has successfully been implemented in certain newborn screening programs to diagnose X-linked adrenoleukodystrophy (ALD)...
February 4, 2024: Journal of Lipid Research
https://read.qxmd.com/read/38285195/identification-of-qtls-associated-with-very-long-chain-fatty-acid-vlcfa-content-via-linkage-mapping-and-bsa-seq-in-peanut
#8
JOURNAL ARTICLE
Xiaomeng Xue, Jianguo Li, Jie Wu, Meiling Hu, Nian Liu, Liying Yan, Yuning Chen, Xin Wang, Yanping Kang, Zhihui Wang, Huifang Jiang, Yong Lei, Chunyu Zhang, Boshou Liao, Dongxin Huai
Three major QTLs qA01, qB04.1 and qB05 for VLCFA content and their corresponding allele-specific markers will benefit peanut low VLCFA breeding, and a candidate gene Arahy.IF1JV3 was predicted. Peanut is a globally significant oilseed crop worldwide, and contains a high content (20%) of saturated fatty acid (SFA) in its seeds. As high level SFA intake in human dietary may increase the cardiovascular disease risk, reducing the SFA content in peanut is crucial for improving its nutritional quality. Half of the SFAs in peanut are very long-chain fatty acids (VLCFA), so reducing the VLCFA content is a feasible strategy to decrease the total SFA content...
January 29, 2024: TAG. Theoretical and Applied Genetics. Theoretische und Angewandte Genetik
https://read.qxmd.com/read/38244676/protective-effect-of-oleic-acid-against-very-long-chain-fatty-acid-induced-apoptosis-in-peroxisome-deficient-cho-cells
#9
JOURNAL ARTICLE
Hanif Ali, Mone Yamanishi, Keigo Sunagawa, Mizuki Kumon, Rumana Yesmin Hasi, Mutsumi Aihara, Ryushi Kawakami, Tamotsu Tanaka
Very long-chain fatty acids (VLCFAs) are degraded exclusively in peroxisomes, as evidenced by the accumulation of VLCFAs in patients with certain peroxisomal disorders. Although accumulation of VLCFAs is considered to be associated with health issues, including neuronal degeneration, the mechanisms underlying VLCFAs-induced tissue degeneration remain unclear. Here, we report the toxic effect of VLCFA and protective effect of C18: 1 FA in peroxisome-deficient CHO cells. We examined the cytotoxicity of saturated and monounsaturated VLCFAs with chain-length at C20-C26, and found that longer and saturated VLCFA showed potent cytotoxicity at lower accumulation levels...
January 18, 2024: Biochimica et Biophysica Acta. Molecular and Cell Biology of Lipids
https://read.qxmd.com/read/38206190/a-genome-wide-association-study-identifies-genes-associated-with-cuticular-wax-metabolism-in-maize
#10
JOURNAL ARTICLE
Liping Xu, Jiaxin Hao, Mengfan Lv, Peipei Liu, Qidong Ge, Sainan Zhang, Jianping Yang, Hongbin Niu, Yiru Wang, Yadong Xue, XiaoDuo Lu, Jihua Tang, Jun Zheng, Mingyue Gou
The plant cuticle is essential in plant defense against biotic and abiotic stresses. To systematically elucidate the genetic architecture of maize (Zea mays L.) cuticular wax metabolism, two cuticular wax-related traits, the chlorophyll extraction rate (CER) and water loss rate (WLR) of 389 maize inbred lines, were investigated and a genome-wide association study (GWAS) was performed using 1.25 million single nucleotide polymorphisms (SNPs). In total, 57 non-redundant quantitative trait loci (QTL) explaining 5...
January 11, 2024: Plant Physiology
https://read.qxmd.com/read/38146202/increased-neurotoxicity-of-high-density-lipoprotein-secreted-from-murine-reactive-astrocytes-deficient-in-a-peroxisomal-very-long-chain-fatty-acid-transporter-abcd1
#11
JOURNAL ARTICLE
Naoki Fujitani, Tomoya Akashi, Masayoshi Saito, Masashi Morita, Takanori So, Kozo Oka
X-linked adrenoleukodystrophy (X-ALD) is a genetic neurodegenerative disorder caused by pathogenic variants in ABCD1, resulting in the accumulation of very-long-chain fatty acids (VLCFAs) in tissues. The etiology of X-ALD is unclear. Activated astrocytes play a pathological role in X-ALD. Recently, reactive astrocytes have been shown to induce neuronal cell death via saturated lipids in high-density lipoprotein (HDL), although how HDL from reactive astrocytes exhibits neurotoxic effects has yet to be determined...
December 25, 2023: Journal of Inherited Metabolic Disease
https://read.qxmd.com/read/38136568/efficacy-of-hdac-inhibitors-in-driving-peroxisomal-%C3%AE-oxidation-and-immune-responses-in-human-macrophages-implications-for-neuroinflammatory-disorders
#12
JOURNAL ARTICLE
Andrea Villoria-González, Bettina Zierfuss, Patricia Parzer, Elisabeth Heuböck, Violetta Zujovic, Petra Waidhofer-Söllner, Markus Ponleitner, Paulus Rommer, Jens Göpfert, Sonja Forss-Petter, Johannes Berger, Isabelle Weinhofer
Elevated levels of saturated very long-chain fatty acids (VLCFAs) in cell membranes and secreted lipoparticles have been associated with neurotoxicity and, therefore, require tight regulation. Excessive VLCFAs are imported into peroxisomes for degradation by β-oxidation. Impaired VLCFA catabolism due to primary or secondary peroxisomal alterations is featured in neurodegenerative and neuroinflammatory disorders such as X-linked adrenoleukodystrophy and multiple sclerosis (MS). Here, we identified that healthy human macrophages upregulate the peroxisomal genes involved in β-oxidation during myelin phagocytosis and pro-inflammatory activation, and that this response is impaired in peripheral macrophages and phagocytes in brain white matter lesions in MS patients...
November 23, 2023: Biomolecules
https://read.qxmd.com/read/38134124/quantifying-resistance-to-very-long-chain-fatty-acid-inhibiting-herbicides-in-amaranthus-tuberculatus-using-a-soilless-assay
#13
JOURNAL ARTICLE
Dylan R Kerr, Jeanaflor Crystal T Concepcion, Seth A Strom, Dean E Riechers
Resistance to preemergence (PRE) soil-applied herbicides, such as inhibitors of very-long-chain fatty acid (VLCFA) elongases, was documented in two waterhemp [Amaranthus tuberculatus (Moq.) J.D. Sauer] populations (SIR and CHR) from Illinois, USA. To limit the spread of resistant weed populations, rapid detection measures are necessary. Soil-based resistance assays are limited by edaphic factors, application timing, variable seeding depth and rainfall amount. Therefore, cost-effective techniques mitigating effects of edaphic factors that are appropriate for small- to large-scale assays are needed...
2023: PloS One
https://read.qxmd.com/read/38116151/genome-wide-identification-and-expression-analysis-of-the-kcs-gene-family-in-soybean-glycine-max-reveal-their-potential-roles-in-response-to-abiotic-stress
#14
JOURNAL ARTICLE
Yujie Gong, Deying Wang, Haojie Xie, Zewei Zhao, Yuyue Chen, Dongxue Zhang, Yexuan Jiao, Mengmeng Shi, Peng Lv, Qi Sha, Jing Yang, Pengfei Chu, Yongwang Sun
Very long chain fatty acids (VLCFAs) are fatty acids with chain lengths of 20 or more carbon atoms, which are the building blocks of various lipids that regulate developmental processes and plant stress responses. 3-ketoacyl-CoA synthase encoded by the KCS gene is the key rate-limiting enzyme in VLCFA biosynthesis, but the KCS gene family in soybean ( Glycine max ) has not been adequately studied thus far. In this study, 31 KCS genes (namely GmKCS1 - GmKCS31 ) were identified in the soybean genome, which are unevenly distributed on 14 chromosomes...
2023: Frontiers in Plant Science
https://read.qxmd.com/read/38092592/fatty-acid-analysis-in-serum-of-patients-with-elevated-lipoprotein-a-and-cardiovascular-disease-undergoing-lipoprotein-apheresis
#15
JOURNAL ARTICLE
Agnieszka Mickiewicz, Joanna Marlęga-Linert, Monika Czapiewska, Marta Marcinkowska, Aleksandra Krzesińska, Agnieszka Kuchta, Marcin Fijałkowski, Marcin Gruchała, Adriana Mika
BACKGROUND: Lipoprotein apheresis (LA) is an extracorporeal treatment that transiently reduces lipoprotein (a) by 60% and leads to an 80-92% reduction in major adverse cardiovascular events. LA has a significant impact on lipid profile in serum of patients with atherosclerotic cardiovascular disease. OBJECTIVE: To investigate the effects of LA on the composition of serum fatty acids (FAs), focusing on those which could have an impact on cardiovascular disease (CVD)...
November 28, 2023: Journal of Clinical Lipidology
https://read.qxmd.com/read/38077449/ipsc-derived-astrocytes-to-model-neuroinflammatory-and-metabolic-responses-in-x-linked-adrenoleukodystrophy
#16
JOURNAL ARTICLE
Parveen Parasar, Navtej Kaur, Jaspreet Singh
X-linked adrenoleukodystrophy (X-ALD) is an inherited metabolic disorder caused by pathogenic variants in the ABCD1 gene, leading to accumulation of saturated very long chain fatty acids (VLCFA) in body fluids and tissues including brain and spinal cord. In the absence of a clear genotype-phenotype correlation the molecular mechanisms of the fatal cerebral adrenoleukodystrophy (cALD) and the milder adrenomyeloneuropathy (AMN) phenotypes remain unknown. Given our previous evidence of role of astrocytes in the neuroinflammatory response in X-ALD we investigated the metabolic and molecular profiles of astrocytes derived from induced pluripotent stem cells (iPSC)...
2023: J Biotechnol Biomed
https://read.qxmd.com/read/38066620/ataxia-with-giant-axonopathy-in-acbd5-deficient-mice-halted-by-adeno-associated-virus-gene-therapy
#17
JOURNAL ARTICLE
Luis Granadeiro, Violeta Enríquez Zarralanga, Ricardo Rosa, Filipa Franquinho, Sofia Lamas, Pedro Brites
Acyl-CoA binding domain containing 5 (ACBD5) is a critical player in handling very long chain fatty acids (VLCFA) on route for peroxisomal β-oxidation. Mutations in ACBD5 lead to the accumulation of VLCFA, and patients present retinal dystrophy, ataxia, psychomotor delay, and a severe leukodystrophy. Using CRISPR/Cas9, we generated and characterized an Acbd5 Gly357* mutant allele. Gly357* mutant mice recapitulated key features of the human disorder, including reduced survival, impaired locomotion and reflexes, loss of photoreceptors, and demyelination...
December 8, 2023: Brain
https://read.qxmd.com/read/38039119/orosomucoid-protein-1-regulation-of-sphingolipid-synthesis-is-required-for-nodulation-in-aeschynomene-evenia
#18
JOURNAL ARTICLE
Nico Nouwen, Marjorie Pervent, Franck El M'Chirgui, Frédérique Tellier, Maëlle Rios, Natasha Horta Araújo, Christophe Klopp, Frédéric Gressent, Jean-François Arrighi
Legumes establish symbiotic interactions with nitrogen-fixing rhizobia that are accomodated in root-derived organs known as nodules. Rhizobial recognition triggers a plant symbiotic signalling pathway that activates two coordinated processes: infection and nodule organogenesis. How these processes are orchestrated in legume species utilizing intercellular infection and lateral root base nodulation remains elusive. Here, we show that Aeschynomene evenia OROSOMUCOID PROTEIN 1 (AeORM1), a key regulator of sphingolipid biosynthesis, is required for nodule formation...
December 1, 2023: Plant Physiology
https://read.qxmd.com/read/38034003/x-linked-adrenoleukodystrophy-and-primary-adrenal-insufficiency
#19
REVIEW
Marco Cappa, Tommaso Todisco, Carla Bizzarri
X-linked adrenoleukodystrophy (X-ALD; OMIM:300100) is a progressive neurodegenerative disorder caused by a congenital defect in the ATP-binding cassette transporters sub-family D member 1 gene (ABCD1) producing adrenoleukodystrophy protein (ALDP). According to population studies, X-ALD has an estimated birth prevalence of 1 in 17.000 subjects (considering both hemizygous males and heterozygous females), and there is no evidence that this prevalence varies among regions or ethnic groups. ALDP deficiency results in a defective peroxisomal β-oxidation of very long chain fatty acids (VLCFA)...
2023: Frontiers in Endocrinology
https://read.qxmd.com/read/38002353/ai-based-homology-modelling-of-fatty-acid-transport-protein-1-using-alphafold-structural-elucidation-and-molecular-dynamics-exploration
#20
JOURNAL ARTICLE
Ranjitha Acharya, Shilpa S Shetty, Gollapalli Pavan, Flama Monteiro, Manne Munikumar, Sriram Naresh, Nalilu Suchetha Kumari
Fatty acid transport protein 1 (FATP1) is an integral transmembrane protein that is involved in facilitating the translocation of long-chain fatty acids (LCFA) across the plasma membrane, thereby orchestrating the importation of LCFA into the cell. FATP1 also functions as an acyl-CoA ligase, catalyzing the ATP-dependent formation of fatty acyl-CoA using LCFA and VLCFA (very-long-chain fatty acids) as substrates. It is expressed in various types of tissues and is involved in the regulation of crucial signalling pathways, thus playing a vital role in numerous physiological and pathological conditions...
November 20, 2023: Biomolecules
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