Genotypic and Phenotypic Characteristics of Pediatric X-Adrenoleukodystrophy in a Chinese Cohort

Authors: Zhang Y, Shi X, Huang J, Wang C, Wu S, Tian G, Jia J, Chen Y

Source: Neuropsychiatric Disease and Treatment, Vol Volume 21, Pp 677-687 (2025)

Subject Terms: abcd1 gene, hematopoietic stem cell transplantation, pediatric patients, very long chain fatty acids, x-adrenoleukodystrophy, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571, Neurology. Diseases of the nervous system, RC346-429

File Description: electronic resource

Relation: https://www.dovepress.com/genotypic-and-phenotypic-characteristics-of-pediatric-x-adrenoleukodys-peer-reviewed-fulltext-article-NDT; https://doaj.org/toc/1178-2021

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Reassessing very long chain fatty acids elevations: Sitosterolemia as a non-peroxisomal cause

Authors: Merve Yoldaş Çelik, Burcu Köşeci, Ezgi Burgaç, Kanay Yararbaş

Source: Molecular Genetics and Metabolism Reports, Vol 42, Iss , Pp 101178- (2025)

Subject Terms: Sitosterolemia, Elevated very long chain fatty acids, VLCFA, False positive VLCFA, Medicine (General), R5-920, Biology (General), QH301-705.5

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Relation: http://www.sciencedirect.com/science/article/pii/S2214426924001319; https://doaj.org/toc/2214-4269

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Normal very long-chain fatty acids level in a patient with peroxisome biogenesis disorders: a case report

Authors: Bita Barazandeh Shirvan, Najmeh Ahangari, Razie Rezaie, Parvaneh Layegh, Ehsan Ghayoor Karimiani, Narges Hashemi, Mehran Beiraghi Toosi

Source: BMC Pediatrics, Vol 24, Iss 1, Pp 1-5 (2024)

Subject Terms: Peroxisome biogenesis disorders, Very long-chain fatty acids (VLCFAs), WES, VUS, PEX6, Pediatrics, RJ1-570

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Relation: https://doaj.org/toc/1471-2431

Access URL: https://doaj.org/article/a0c587b79d8e4370b06c8a859c922065

From gene to therapy: a review of deciphering the role of ABCD1 in combating X-Linked adrenoleukodystrophy

Authors: Xinxin Zuo, Zeyu Chen

Source: Lipids in Health and Disease, Vol 23, Iss 1, Pp 1-11 (2024)

Subject Terms: X-linked adrenoleukodystrophy (X-ALD), Very-long-chain fatty acids (VLCFAs), Genetic mutations, ABCD1, Neurological decline, Gene therapy, Nutritional diseases. Deficiency diseases, RC620-627

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Relation: https://doaj.org/toc/1476-511X

Access URL: https://doaj.org/article/d426e0bad892454db8a1a4d32a77959a

Development of a rabbit model for adrenoleukodystrophy: A pilot study on gene therapy using rAAV9

Authors: Zhou, Xiaoya ^{1, 2, 12}, Ma, Chui-Yan ^{3, 12}, Zhang, Xiaoxian ^{1, 2, 12}, Xu, Xianchuan ², Duan, Fuyu ^{1, 2}, Kou, Meng ^{1, 2}, Liu, Hongsheng ⁵, Zeng, Liang ⁶, Guo, Liyan ^{1, 2}, Chen, Shaoxiang ^{1, 2}, Chen, Li ^{1, 2}, Li, Ziyue ^{1, 2}, Luo, Jie ^{1, 2}, Wu, Jieying ¹, Li, Zhejin ⁷, Li, Zhanjun ⁷, Sui, Tingting ⁷, Yuan, Ping ⁸, Lin, Zhijian ⁹, Chen, Hao ¹⁰, Lai, Liangxue ^{7, 11}, Lian, Qizhou ^{1, 2, 3, 4, ∗}

Source: In Molecular Therapy - Nucleic Acids 11 March 2025 36(1)

The ELOVL proteins: Very and ultra long-chain fatty acids at the crossroads between metabolic and neurodegenerative disorders

Authors: Ferrero, Enza ^{a, 1}, Vaz, Frédéric M. ^{b, c, d, ⁎⁎, 1}, Cheillan, David ^{e, f}, Brusco, Alfredo ^{g, h}, Marelli, Cecilia ^{i, j, ⁎}

Source: In Molecular Genetics and Metabolism March 2025 144(3)

VAP27-1 interacts with KCS6 and CER2 to facilitate the biosynthesis of very- long-chain fatty acids

Authors: Wang, Haiyan ^{a, b}, Li, Yifan ^{a, b}, Wang, Qinyao ^{a, b}, Wu, Mengxia ^{a, b}, Wang, Ruiyuan ^b, Han, Xinran ^b, Liu, Lin ^b, Liu, Ting ^{a, b}, Shi, Chunmei ^{a, b}, Zhong, Linlin ^{a, b}, Zhang, Hongyan ^{a, b}, Cheng, Yunjiang ^{a, b, c}, Wang, Pengwei ^{a, b}, Qu, Xiaolu ^{a, b, ⁎}

Source: In Plant Science June 2025 355

Genome-wide identification and molecular evolution of elongation family of very long chain fatty acids proteins in Cyrtotrachelus buqueti

Authors: Chun Fu, Ting Yang, Hong Liao, YuLing Huang, HanYu Wang, WenCong Long, Na Jiang, YaoJun Yang

Source: BMC Genomics, Vol 25, Iss 1, Pp 1-22 (2024)

Subject Terms: Cyrtotrachelus buqueti, Elongation family of very long chain fatty acids proteins, Genome-wide identification, Molecular evolution, Evolutionary analysis, Biotechnology, TP248.13-248.65, Genetics, QH426-470

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Relation: https://doaj.org/toc/1471-2164

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Genome-Wide Identification of β-Ketoacyl CoA Synthase Gene Family in Melon (Cucumis melo L.) and Its Expression Analysis in Autotoxicity, Saline-Alkali, and Microplastic Exposure Environments

Authors: Lizhen Zhang, Mingcheng Wang, Xianhuan Tang, Xinyue Yang, Zhizhong Zhang, Jinghua Wu

Source: Current Issues in Molecular Biology, Vol 47, Iss 3, p 195 (2025)

Subject Terms: β-ketoacyl CoA synthase gene, cucurbits, molecular phylogeny, stress response gene, very-long-chain fatty acids, Biology (General), QH301-705.5

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Relation: https://www.mdpi.com/1467-3045/47/3/195; https://doaj.org/toc/1467-3037; https://doaj.org/toc/1467-3045

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An in vitro and in vivo efficacy evaluation of gene therapy candidate SBT101 in mouse models of adrenomyeloneuropathy and in NHPs

Authors: Vasireddy, Vidyullatha ^{1, 11}, Maguire, Casey A. ^{2, 3}, Anderson, David W. ^{1, 11}, Ng, Carrie ^{2, 3}, Gong, Yi ^{2, 3}, Eichler, Florian ^{2, 3}, Fourcade, Stéphane ^{4, 5}, Guilera, Cristina ^{4, 5}, Onieva, Andrea ^{6, 7}, Sanchez, Angela ^{6, 7}, Leal-Julià, Marc ^{6, 7}, Verdés, Sergi ^{6, 7}, Dijkstra, Inge M.E. ⁹, Kemp, Stephan ⁹, Park, HongGeun ^{1, 12}, Lutz, Tiffany ^{1, 13}, Clark, Sean W. ^{1, 14}, Bosch, Assumpció ^{6, 7, 8}, Pujol, Aurora ^{4, 5, 10}, Kozarsky, Karen ^{1, 15, ∗}

Source: In Molecular Therapy - Methods & Clinical Development 12 December 2024 32(4)

The Absence of a Very Long Chain Fatty Acid (VLCFA) in Lipid A Impairs Agrobacterium fabrum Plant Infection and Biofilm Formation and Increases Susceptibility to Environmental Stressors

Authors: Iwona Komaniecka, Kamil Żebracki, Andrzej Mazur, Katarzyna Suśniak, Anna Sroka-Bartnicka, Anita Swatek, Adam Choma

Source: Molecules, Vol 30, Iss 5, p 1080 (2025)

Subject Terms: Agrobacterium fabrum, lipid A, very long chain fatty acids, mass spectrometry, biofilm, FTIR spectroscopy, Organic chemistry, QD241-441

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Relation: https://www.mdpi.com/1420-3049/30/5/1080; https://doaj.org/toc/1420-3049

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Magnetic resonance imaging enhancement of spinal nerve roots in a boy with X-linked adrenoleukodystrophy before diagnosis of chronic inflammatory demyelinating polyneuropathy

Authors: Derryl Miller, MD, Laurence Walsh, MD, Lisa Smith, MD, Nucharin Supakul, MD, Chang Ho, MD, Toshihiro Onishi, MD

Source: Radiology Case Reports, Vol 19, Iss 1, Pp 493-498 (2024)

Subject Terms: Adrenoleukodystrophy, Very long chain fatty acids, ATP binding cassette subfamily D member 1, Chronic inflammatory demyelinating polyneuropathy, Medical physics. Medical radiology. Nuclear medicine, R895-920

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Relation: http://www.sciencedirect.com/science/article/pii/S1930043323008026; https://doaj.org/toc/1930-0433

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Genotoxicity evaluation of a fish oil concentrate containing Very Long Chain Fatty Acids

Authors: Derek Tobin, Harald Svensen, Iren Stoknes, Michael Dornish

Source: Toxicology Reports, Vol 11, Iss , Pp 249-258 (2023)

Subject Terms: Toxicity, Omega-3, Very-long-chain-fatty-acids, VCLFA, Safety, EPAX EVOLVE, Toxicology. Poisons, RA1190-1270

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Relation: http://www.sciencedirect.com/science/article/pii/S2214750023001014; https://doaj.org/toc/2214-7500

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The impact of the GLOSSY2 and GLOSSY2-LIKE BAHD-proteins in affecting the product profile of the maize fatty acid elongase

Authors: Liza Esther Alexander, Dirk Winkelman, Kenna E. Stenback, Madison Lane, Katelyn R. Campbell, Elysse Trost, Kayla Flyckt, Michael A. Schelling, Ludmila Rizhsky, Marna D. Yandeau-Nelson, Basil J. Nikolau

Source: Frontiers in Plant Science, Vol 15 (2024)

Subject Terms: fatty acid elongase, very long chain fatty acids, GLOSSY2, GLOSSY2-LIKE, BAHD proteins, maize, Plant culture, SB1-1110

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Relation: https://www.frontiersin.org/articles/10.3389/fpls.2024.1403779/full; https://doaj.org/toc/1664-462X

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Brainstem dominant form of X‐linked adrenoleukodystrophy with a novel ABCD1 missense variant: A case report and literature review

Authors: Yulai Kang, Lu Guo, Zhuo Min, Lei Zhang, Lili Zhang, Chunhua Tang

Source: Molecular Genetics & Genomic Medicine, Vol 12, Iss 7, Pp n/a-n/a (2024)

Subject Terms: ABCD1, brainstem, MRI, very long‐chain fatty acids, X‐linked adrenoleukodystrophy, Genetics, QH426-470

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Relation: https://doaj.org/toc/2324-9269

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Four-dimensional lipidomics profiling in X-linked adrenoleukodystrophy using trapped ion mobility mass spectrometry

Authors: Yorrick R.J. Jaspers, Sven W. Meyer, Mia L. Pras-Raves, Inge M.E. Dijkstra, Eric J.M. Wever, Adrie D. Dane, Jan-Bert van Klinken, Gajja S. Salomons, Riekelt H. Houtkooper, Marc Engelen, Stephan Kemp, Michel Van Weeghel, Frédéric M. Vaz

Source: Journal of Lipid Research, Vol 65, Iss 6, Pp 100567- (2024)

Subject Terms: 4D-Lipidomics, parallel accumulation serial fragmentation, very-long-chain fatty acids, trapped ion mobility spectrometry, adrenoleukodystrophy, Biochemistry, QD415-436

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Relation: http://www.sciencedirect.com/science/article/pii/S0022227524000725; https://doaj.org/toc/0022-2275

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Peripheral peroxisomal β-oxidation engages neuronal serotonin signaling to drive stress-induced aversive memory in C. elegans

Authors: Tsai, Shang-Heng ^{1, 2}, Wu, Yu-Chun ^{1, 2}, Palomino, Diana Fajardo ³, Schroeder, Frank C. ³, Pan, Chun-Liang ^{1, 4, ∗}

Source: In Cell Reports 23 April 2024 43(4)

Breeding, Genetics and Safety Production of Dairy Cattle

Contributors: Chen, Zhi, Li, Cong

Subject Terms: mammary epithelial cells, milk-fat synthesis, miR-125a, SAA1, MAC-T, cow, milk production, heard health status, APPs, SAA, Lycium barbarum polysaccharide, bovine mammary epithelial cells, E. coli, MAPK signaling, SOCS3, peroxisome, very long-chain fatty acids, beta-oxidation, cofilin-1, cofilin-2, expression pattern, DNA methylation, myoblast differentiation, m6A methylation, lipid metabolism, molecular mechanism, detection tools, Escherichia coli, drug resistance, bovine mastitis, β-lactams, DDIT3, genetic effect, milk production traits, apoptosis, dairy cattle, hereditary diseases, breeds, mutations, alleles, genetic-load classification, Holstein cattle, fertility, gene expression, ovarian follicle, haplotype, miR-29, GPAM, milk fat, cow mammary cells, Research and information: general, Biology, life sciences, Technology, Engineering, Agriculture, Industrial processes

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Access URL: https://directory.doabooks.org/handle/20.500.12854/113866
https://mdpi.com/books/pdfview/book/7707

Role and Function of Peroxisomes in Neuroinflammation

Authors: Chinmoy Sarkar, Marta M. Lipinski

Source: Cells, Vol 13, Iss 19, p 1655 (2024)

Subject Terms: peroxisomes, neuroinflammation, peroxisomal disorders, very-long-chain fatty acids, ether phospholipids, Cytology, QH573-671

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Relation: https://www.mdpi.com/2073-4409/13/19/1655; https://doaj.org/toc/2073-4409

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Adrenoleukodystrophy: A Rare Clinical Scenario

Authors: Antara Kunwar, Jitendriya Biswal

Source: Indian Journal of Private Psychiatry, Vol 18, Iss 1, Pp 45-47 (2024)

Subject Terms: abcd1 gene, adrenoleukodystrophy, case report, very long-chain fatty acids, Psychiatry, RC435-571

File Description: electronic resource

Relation: https://doaj.org/toc/2319-5363; https://doaj.org/toc/2583-4010

Access URL: https://doaj.org/article/a106b49af97d478c99b03c985d139462