Biochemistry and Biophysics Reports最新文献

{"title":"\"Protective effects of artichoke extract and Bifidobacterium longum on male infertility in diabetic rats\"","authors":"Zahra Ansari ,&nbsp;Mohammad Hasan Maleki ,&nbsp;Fatemeh Roohy ,&nbsp;Zahra Ebrahimi ,&nbsp;Mesbah Shams ,&nbsp;Pooneh Mokaram ,&nbsp;Zahra Zamanzadeh ,&nbsp;Zahra Hosseinzadeh ,&nbsp;Farhad Koohpeyma ,&nbsp;Sanaz Dastghaib","doi":"10.1016/j.bbrep.2024.101834","DOIUrl":"10.1016/j.bbrep.2024.101834","url":null,"abstract":"<div><h3>Background</h3><div>Diabetes is a major global health concern and plays a significant role in male infertility and hormonal abnormalities by altering the tissue structure of spermatogenic tubes and decreasing the number of spermatogonia. This study investigated the effect of artichoke (<em>Cynara scolymus</em> L) hydroalcoholic extract and <em>Bifidobacterium longum</em> probiotic on sexual hormones, oxidative stress, apoptosis pathway, and histopathological changes in testicular tissues of diabetic rats to find an adjuvant therapy to manage the infertility complications of diabetes.</div></div><div><h3>Methods</h3><div>In this experiment, 96 male-rats were randomly selected from eight groups. Control, Sham (normal saline), DM group (IP injected with 60 mg/kg STZ), Cynara (400 mg/kg hydroalcoholic extract of Cynara scolymus L), BBL (received 1 × 10⁹ CFU/ml/day Bifidobacterium longum), DM + Cynara, DM + BBL, and DM + Cynara + BBL groups. After 48 days of orally gavage, serum level of FBS (fasting blood sugar), Malondi-aldehyde (MDA), Total-Anti-Oxidant Capacity (TAC), FSH (Follicle-stimulating hormone), LH (Luteinizing hormone), Testosterone, Testis mRNA-expressions of <em>Protamin</em> (prm1), <em>BCL2</em>, and <em>Caspase-9</em> genes, as well as stereological changes were measured.</div></div><div><h3>Results</h3><div>In comparison to the diabetic group, the hydroalcoholic extract of <em>Cynara scolymus</em> L combined with the probiotic <em>Bifidobacterium longum</em> resulted in a substantial decrease in FBS (p &lt; 0.001) and MDA(p &lt; 0.05) concentrations, and the expression of the Caspase-9 gene (1.33-fold change). In addition, serum levels of TAC, LH, FSH, Testosterone were significantly increased (p &lt; 0.05). mRNA expression of protamine (p = 0.016) and BCL2 (0.72-fold change) were detected. Furthermore, in comparison with diabetic rats, the <em>Cynara scolymus L</em>-and <em>Bifidobacterium longum</em>-treated groups showed a significant increase in the number of sexual lineage cells, total weight, sperm count, motility, normal morphology, volume of the testis, and volume and length of seminiferous tubules (p &lt; 0.05).</div></div><div><h3>Conclusion</h3><div>The findings demonstrated that Cynara scolymus L extract and Bifidobacterium longum supplement had great therapeutic potential, including antioxidant, anti-apoptotic, anti-diabetic, fertility index improvement, and sex hormone modulators.</div></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001985/pdfft?md5=c8a22fb0929f97ffc9d6717e7bac814b&pid=1-s2.0-S2405580824001985-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142311531","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unveiling functionality and conducting two-sample mendelian randomization on WGCNA-identified oxidative stress-related hub genes in metabolic dysfunction-associated fatty liver disease","authors":"Qian Zhu ,&nbsp;Jiaqi Liu ,&nbsp;Wuxuan Mei ,&nbsp;Changchun Zeng","doi":"10.1016/j.bbrep.2024.101829","DOIUrl":"10.1016/j.bbrep.2024.101829","url":null,"abstract":"<div><div>Metabolic dysfunction-associated fatty liver disease (MAFLD) shows accelerated development under the impact of oxidative stress (OS). There is an imperative to identify OS-related biomarkers in MAFLD and explore their potential mechanistic insights. The objective of this study was to identify OS-related biomarkers in MAFLD and explore their potential mechanisms. DEG analysis was performed using GSE17470 and GSE24807 datasets. An investigative exploration utilizing WGCNA was executed to elucidate hub OS-related genes. The intersection of OS-related hub genes identified by WGCNA and DEGs was systematically employed for thorough analyses. A mendelian randomization (MR) study examined the causal effect of C-reactive protein (CRP) on MAFLD. 59 OS-related DEGs were identified in MAFLD. WGCNA revealed 100 OS-related hub genes in MAFLD. Sixteen OS-related genes have been delineated as critical components in MAFLD. Enrichment analyses, employing GO and KEGG pathways, revealed pathways enriched with these genes. Following PPI analyses, the highest-ranking ten hub genes demonstrating abnormal expression were determined. Ultimately, a two-sample MR analysis demonstrated a causal link between the hub gene CRP and the occurrence of MAFLD. In this study, we harnessed WGCNA to formulate a co-expression network and identified hub OS-related DEGs in MAFLD. Additionally, the hub gene CRP exhibited a significant correlation with the predisposition to MAFLD. These findings offer innovative perspectives on the applications of OS-associated genes in individuals afflicted with MAFLD.</div></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001936/pdfft?md5=01913b6daf9306d3181352a0065940b5&pid=1-s2.0-S2405580824001936-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142311013","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epigenetic regulation of the human GDAP1 gene","authors":"Kaja Karaś,&nbsp;Joanna Pastwińska,&nbsp;Anna Sałkowska,&nbsp;Iwona Karwaciak,&nbsp;Marcin Ratajewski","doi":"10.1016/j.bbrep.2024.101827","DOIUrl":"10.1016/j.bbrep.2024.101827","url":null,"abstract":"<div><p>Mutations in the ganglioside-induced differentiation-associated protein 1 (<em>GDAP1</em>) gene are linked to Charcot–Marie–Tooth (CMT) disease, a hereditary neurodegenerative condition. The protein encoded by this gene is involved in mitochondrial fission and calcium homeostasis. Recently, GDAP1 has also been implicated in the survival of patients with certain cancers. Despite its significant role in specific cellular processes and associated diseases, the mechanisms regulating <em>GDAP1</em> expression are largely unknown. Here, we show for the first time that methylation of the CpG island in the proximal promoter of the <em>GDAP1</em> gene inhibits its activity. Treating cells with low <em>GDAP1</em> expression using methyltransferase and HDAC inhibitors induced the expression of this gene and its encoded protein. This induction was associated with promoter demethylation and increased association of acetylated histones with the <em>GDAP1</em> promoter. Thus, we identified a mechanism that could be used to manipulate <em>GDAP1</em> expression.</p></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001912/pdfft?md5=f9fd08807cb4fb17253e8d0588917795&pid=1-s2.0-S2405580824001912-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142239098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of alveolar macrophages in viral respiratory infections and their therapeutic implications","authors":"Atefe Panahipoor Javaherdehi ,&nbsp;Somayyeh Ghanbari ,&nbsp;Pooya Mahdavi ,&nbsp;Alireza Zafarani ,&nbsp;Mohammad Hossein Razizadeh","doi":"10.1016/j.bbrep.2024.101826","DOIUrl":"10.1016/j.bbrep.2024.101826","url":null,"abstract":"<div><p>Alveolar macrophages are pivotal components of the lung's innate immune defense against respiratory virus infections. Their multifaceted role spans from viral clearance to modulation of immune responses, making them essential players in shaping disease outcomes. In this comprehensive review collection, we look into the intricate interplay between Alveolar macrophages and various respiratory viruses, shedding light on their dynamic contributions to immune resilience. From influenza to respiratory syncytial virus, Alveolar macrophages emerge as sentinels of the airways, actively participating in viral detection and initiating rapid antiviral responses. Their ability to recognize viral pathogens triggers a cascade of events, including cytokine and chemokine production that guides the recruitment and activation of immune effectors. Furthermore, Alveolar macrophages impact the fate of adaptive immune responses by modulating the activation of T lymphocytes and the secretion of key cytokines. These reviews encompass a range of insights, including the regulation of inflammasome activation, the influence of Alveolar macrophages on cytokine dysregulation, and their role in preventing secondary bacterial pneumonia post-infection. Collectively, they highlight the significance of Alveolar macrophages in preserving pulmonary integrity and immune homeostasis during viral challenges.</p></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001900/pdfft?md5=b06597a378506d5f191363dedadffbb0&pid=1-s2.0-S2405580824001900-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142239096","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exogenous interactome analysis of bovine viral diarrhea virus-host using network based-approach and identification of hub genes and important pathways involved in virus pathogenesis","authors":"Seyedeh Elham Rezatofighi","doi":"10.1016/j.bbrep.2024.101825","DOIUrl":"10.1016/j.bbrep.2024.101825","url":null,"abstract":"<div><p>Bovine viral diarrhea (BVD) is one of the most important diseases in livestock, caused by BVD virus (BVDV). During the pathogenesis of the virus, many interactions occur between host and viral proteins. Studying these interactions can help better understand the pathogenesis of the virus, identify putative functional proteins, and find new treatment and prevention strategies. To this aim, a BVDV-host protein-protein interaction (PPI) network map was constructed using Cytoscape and analyzed with cytoHubba, Kyoto Encyclopedia of Genes and Genomics (KEGG), Gene Ontology (GO), and Protein Analysis Through Evolutionary Relationships (PANTHER). N_pro with 125 connections had the greatest number of interactions with host proteins. <em>CD46</em>, <em>EEF</em>-2, and <em>TXN</em> genes were detected as hub genes using different ranking algorithms in cytoHubba. BVDV interactions with its host mainly focus on targeting translation, protein synthesis, and cellular metabolism pathways. Different classes of proteins including translational proteins, nucleic acid metabolism proteins, metabolite interconversion enzymes, and protein-modifying enzymes are affected by BVDV. These findings improve our understanding of the effects of the virus on the cell. Hub genes and key pathways identified in the present study can serve as targets for novel BVDV prevention or treatment strategies.</p></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001894/pdfft?md5=6c9ef034d78f7d1328f85fc1eb4ce223&pid=1-s2.0-S2405580824001894-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142239097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development of specific anti-mouse atypical chemokine receptor 4 monoclonal antibodies","authors":"Miu Hirose,&nbsp;Hiroyuki Suzuki,&nbsp;Rena Ubukata,&nbsp;Tomohiro Tanaka,&nbsp;Mika K. Kaneko,&nbsp;Yukinari Kato","doi":"10.1016/j.bbrep.2024.101824","DOIUrl":"10.1016/j.bbrep.2024.101824","url":null,"abstract":"<div><p>Leukocyte migration is an essential function of innate and adaptive immune responses. Chemokines and their receptors control the migration system. The abundance of chemokines is controlled by atypical chemokine receptors (ACKRs), chemokine receptor-like molecules that do not couple to the G protein signaling pathways. Among them, ACKR4 regulates dendritic cell migration by controlling the ligands and is involved in tumor development in mouse models. Because no anti-mouse ACKR4 (mACKR4) monoclonal antibody (mAb) for flow cytometry has been reported, this study aimed to develop a novel mAb for mACKR4. Among the established anti-mACKR4 mAbs, A₄Mab-1 (rat IgG_2b, kappa), A₄Mab-2 (rat IgG_2b, kappa), and A₄Mab-3 (rat IgG_2b, kappa) recognized mACKR4-overexpressed Chinese hamster ovary-K1 (CHO/mACKR4) by flow cytometry. The dissociation constant (<em>K</em>_D) values of A₄Mab-1, A₄Mab-2, and A₄Mab-3 for CHO/mACKR4 were determined as 6.0 × 10⁻⁹ M, 1.3 × 10⁻⁸ M, and 1.7 × 10⁻⁹ M, respectively. Furthermore, A₄Mab-1 and A₄Mab-2 could detect mACKR4 by western blotting. These results indicated that A₄Mab-1, A₄Mab-2, and A₄Mab-3 help to detect mACKR4 by flow cytometry and western blotting and obtain the proof of concept in preclinical models.</p></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001882/pdfft?md5=df1bec1ed4b9eaea11a06516e0feac67&pid=1-s2.0-S2405580824001882-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142150194","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Oxyresveratrol reduces lipopolysaccharide-induced inflammation and oxidative stress through inactivation of MAPK and NF-κB signaling in brain endothelial cells","authors":"Yan Zhou ,&nbsp;Qiaowen Deng ,&nbsp;Chi Teng Vong ,&nbsp;Haroon Khan ,&nbsp;Wai San Cheang","doi":"10.1016/j.bbrep.2024.101823","DOIUrl":"10.1016/j.bbrep.2024.101823","url":null,"abstract":"<div><p>Inflammatory responses and oxidative stress damage the integrity of the blood-brain barrier (BBB), which is a primary pathological modulator of neurodegenerative diseases. Brain endothelial cells are crucial components of BBB. In the present study, the effect of oxyresveratrol on lipopolysaccharide (LPS)-induced brain endothelial (bEnd.3) cells was assessed. Our results showed that oxyresveratrol diminished protein expressions of inducible nitric oxide synthase (iNOS) and adhesion molecules including intercellular adhesion molecule (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1), nitric oxide (NO) production, and proinflammatory cytokines such as interleukin-6 (IL-6) and tumor necrosis factor (TNF-α) in LPS-elicited bEnd.3 cells. These anti-inflammatory effects were mediated through suppressing nuclear factor-kappa B (NF-κB) and mitogen-activated protein kinase (MAPK) signaling pathways. In addition, we found that oxyresveratrol reduced reactive oxygen species (ROS) levels. To conclude, the current results demonstrated the protective role of oxyresveratrol against LPS-induced inflammation and oxidative stress in bEnd.3 cells, suggesting its potential effect for mitigating neurodegenerative and cerebrovascular diseases.</p></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001870/pdfft?md5=4f36cbea5e641469209a5eaea1632a17&pid=1-s2.0-S2405580824001870-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142150193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Identifying molecular targets for modulating carotenoid accumulation in rice grains","authors":"Rakshana Palaniswamy,&nbsp;Rohit Kambale,&nbsp;Vignesh Mohanavel,&nbsp;Veera Ranjani Rajagopalan,&nbsp;Sudha Manickam,&nbsp;Raveendran Muthurajan","doi":"10.1016/j.bbrep.2024.101815","DOIUrl":"10.1016/j.bbrep.2024.101815","url":null,"abstract":"<div><p>Carotenoids are potential antioxidants offering extensive human health benefits including protection against chronic diseases. Augmenting the supply of health-benefiting compounds/metabolites through dietary supplements is the most sustainable way for a healthy life. Our study compares the traditional rice cultivar Kavuni and the white rice variety ASD 16. RNA-Seq analysis was carried out in the maturing panicles of Kavuni, which are enriched with antioxidants such as the therapeutic carotenoid lutein, polyphenols, and anthocyanins, along with “ASD 16”, a popularly eaten white rice variety, to elucidate the molecular networks regulating accumulation of health benefiting compounds. Systematic analysis of transcriptome data identified preferential up-regulation of carotenoid precursors (<em>OsDXS</em>, <em>OsGGPS</em>) and key carotenoid biosynthetic genes (<em>OsPSY1</em>, <em>OsZ-ISO</em>) in the maturing grains of Kavuni. Our study also identified enhanced expression of <em>OsLYC-E</em>, <em>OsCYP97A,</em> and <em>OsCYP97C</em> transcripts involved in the alpha-carotenoid biosynthetic pathway and thereby leading to elevated lutein content in the grains of Kavuni. Kavuni grains showed preferential down-regulation of negative regulators of carotenoid metabolism viz., AP2 and HY5 and preferential up-regulation of positive modulators of carotenoid metabolism viz., <em>Orange</em>, <em>OsDjB7,</em> and <em>OsSET29</em>, thus creating a favorable molecular framework for carotenoid accumulation. Our study has unearthed valuable gene control points for precise manipulation of carotenoid profiles through CRISPR-based gene editing in rice grains. Perturbation of carotenoid biosynthesis holds unprecedented potential for the rapid development of the next generation of ‘Golden rice’.</p></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001791/pdfft?md5=9c44fab5940a2f799c998f97f4af1b36&pid=1-s2.0-S2405580824001791-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142150195","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Asparagus racemosus silver chloride nanoparticles and Kaempferia rotunda mediated silver/silver chloride nanoparticles inhibit human hepatocellular and lung cancer cell lines","authors":"Syed Rashel Kabir ,&nbsp;Mohammad Taufiq Alam ,&nbsp;Md Belal Uddin","doi":"10.1016/j.bbrep.2024.101818","DOIUrl":"10.1016/j.bbrep.2024.101818","url":null,"abstract":"<div><p>Recently, we have reported that biogenic silver/silver chloride nanoparticles from <em>Asparagus racemosus</em> (<em>A. racemosus</em>-AgCl-NPs) and <em>Kaempferia rotunda</em> (<em>K. rotunda</em>-Ag/AgCl-NPs) inhibited different cancer cells by inducing apoptosis and several genes alteration. Here for the first time, we assessed the effects of these two nanoparticles on human lung (A549) and hepatocellular (SMMC-7721) carcinoma cell lines. <em>A. racemosus-</em>AgCl-NPs and <em>K. rotunda</em>-Ag/AgCl-NPs inhibited A549 cell growth with IC₅₀ values of 22.7 and 59.7 μg/ml and the calculated IC₅₀ values for SMMC-7721 cell were 89.3 and 126.3 μg/ml, respectively. <em>A. racemosus</em>-AgCl-NPs exerted higher cytotoxicity against HEK293T cells than doxorubicin and <em>K. rotunda</em>-Ag/AgCl-NPs. Both the nanoparticles induced apoptosis in A549 and SMMC-7721 cell lines. A significant rise of early apoptotic cells and late apoptotic cells was found for A549 cells after treatment with <em>A. racemosus</em>-AgCl-NPs and stained with FITC-annexin V/PI. Apoptosis in A549 cells was further confirmed by monitoring the alteration of the expression level of several genes using real-time PCR and cell cycle arrest by flowcytometry after treatment with <em>A. racemosus</em>-AgCl-NPs. The expression of STAT-3, TNFα, and EGFR genes was decreased with the increase of caspase-8, FAS, and FADD gene expression. G₂/M cell cycle phase was arrested after treatment of A549 cells with <em>A. racemosus</em>-AgCl-NPs.</p></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001821/pdfft?md5=b85d7f30e299f375490840e9faab62f5&pid=1-s2.0-S2405580824001821-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142150196","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Altered fatty acid distribution in lysosome-associated membrane protein-2 deficient mice","authors":"Ziming Xu ,&nbsp;Shoji Notomi ,&nbsp;Guannan Wu ,&nbsp;Yosuke Fukuda ,&nbsp;Yusuke Maehara ,&nbsp;Masatoshi Fukushima ,&nbsp;Yusuke Murakami ,&nbsp;Masatomo Takahashi ,&nbsp;Yoshihiro Izumi ,&nbsp;Koh-Hei Sonoda","doi":"10.1016/j.bbrep.2024.101822","DOIUrl":"10.1016/j.bbrep.2024.101822","url":null,"abstract":"<div><p>Lysosome-associated membrane protein-2 (LAMP2) deficiency causes the human Danon disease and represents a lysosomal dysfunction because of its pivotal role in regulating autophagy and lysosome biogenesis. LAMP2-deficient mice exhibit a spectrum of phenotypes, including cardioskeletal myopathy, mental retardation, and retinopathy, similar to those observed in patients with Danon disease. Its pathology is thought to involve altered energy metabolism and lipid dysregulation; however, the lipidomic profiles of LAMP2-deficient animals have not been investigated. In this study, we investigated lipid alterations in LAMP2 KO mice tissues, including those of the liver, plasma, and retina, using liquid chromatography-mass spectrometry. Our results revealed significantly increased free fatty acid (FFA) levels and decreased in triglyceride (TG) levels in LAMP2 KO liver tissues at three and six months. Phosphatidylcholine (PC) and phosphatidylethanolamine (PE) species significantly decreased in LAMP2 KO mice livers at six months. Similarly, plasma TG and PC/PE levels decreased in LAMP2 KO mice. In contrast, plasma FFA levels were significantly lower in LAMP2 KO mice. Retina FFA levels were elevated in LAMP2 KO mice, accompanied by a partial decrease in PC/PE at six months. In summary, FFA levels increased in several tissues but not in the LAMP2 KO mice plasma, suggesting the potential consumption of FFA as an energy source in the peripheral tissues. The depletion of TG and PC/PE accelerated with age, suggesting an underlying age-dependent energy crisis condition. Our findings underscore the dysregulated distribution of fatty acids in LAMP2-deficient animals and provide new mechanistic insights into the pathology of Danon disease.</p></div>","PeriodicalId":8771,"journal":{"name":"Biochemistry and Biophysics Reports","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2405580824001869/pdfft?md5=b3a75aa82ec93056480dab7bade430a1&pid=1-s2.0-S2405580824001869-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142150197","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}