Advancing the Biochemical Understanding of Maple Syrup Urine Disease and the Impact of Liver Transplantation: A Pilot Study DOI
Sotirios P. Fortis, Abby Grier, Julie A. Reisz

и другие.

Journal of Proteome Research, Год журнала: 2025, Номер unknown

Опубликована: Апрель 15, 2025

Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic disorder causing impaired branched-chain amino acid (BCAA) catabolism and systemic dysregulation. MSUD has an incidence of approximately 1 in 185,000 U.S. births, with much higher prevalence the Mennonite communities (up to 400 live births due c.1312T > A p.Tyr438Asn BCKDHA founder mutation). Using multiomic approach integrating metabolomics, lipidomics, proteomics, we analyzed blood samples from three patients on BCAA-restricted diet (MSUDDR), two who received liver transplants (MSUDLT), six healthy controls. Gene ontology analysis integrated omics data confirmed imbalances MSUD, highlighting increases glycolysis, oxidative phosphorylation, purine metabolism. Lipidomic revealed disruptions sphingolipids lysophosphatidylcholines, affecting signaling membrane integrity. Liver transplantation corrected some abnormalities, but key metabolites proteins remained altered. Proteomic significant alterations redox homeostasis, energy metabolism, cytoskeletal organization partial recovery post-transplantation. Post-translational modifications indicated ongoing stress immune activation MSUDLT group. Elevated levels l-isoleucine, l-valine, their ketoacids persisted post-transplant, correlating lipid remodeling, protein folding. These findings provide insights into MSUD-associated dysfunction highlight potential therapeutic targets.

Язык: Английский

Metabolomic Characteristics of Aqueous Humor in Wet Age-Related Macular Degeneration and the Impact of Anti-VEGF Treatment DOI Creative Commons
Yijing Chen, Xiaoya Zhang, Yanli Zhang

и другие.

Investigative Ophthalmology & Visual Science, Год журнала: 2025, Номер 66(2), С. 37 - 37

Опубликована: Фев. 12, 2025

Purpose: To explore the alterations in metabolites of wet age-related macular degeneration (wAMD) by conducting metabolomics aqueous humor from patients with wAMD and to assess potential effects anti-vascular endothelial growth factor (anti-VEGF) on these metabolites. Methods: Metabolomic analysis was performed 30 receiving anti-VEGF treatments 20 controls, via ultra-high performance liquid chromatography tandem mass spectrometry. The samples collected untreated were classified as pre-wAMD group. Accordingly, one treatment designated post-wAMD Individuals further into responders nonresponders according their reaction treatment. Principal component analysis, hierarchical cluster Kyoto Encyclopedia Genes Genomes annotation enrichment subsequently performed. Machine learning receiver operating characteristic curve analyses used analyze vital Results: Among 1001 verified humor, 306 compounds separated control group, whereas 68 differentiated pre-wAMD. Enrichment metabolic pathways noted ABC transporters, thiamine metabolism, glycerophospholipid mammalian target rapamycin signaling pathway tyrosine so on. curves suggested that δ-valerolactam could not only distinguish between but also differentiate Changes acylcarnitine observed wAMD. Conclusions: There noticeable involving many are associated pathway. It is possible can be applied a biomarker

Язык: Английский

Процитировано

0

Bioactive lipid signaling and lipidomics in macrophage polarization: Impact on inflammation and immune regulation DOI Creative Commons
Juan Pablo Rodríguez, Javier Casas, Marı́a A. Balboa

и другие.

Frontiers in Immunology, Год журнала: 2025, Номер 16

Опубликована: Фев. 14, 2025

Macrophages, crucial innate immune cells, defend against pathogens and resolve inflammation, maintaining tissue balance. They perform phagocytosis, present antigens to T bond adaptive immunity through various activation states. Classical is associated with Th1 responses interferon γ production, while alternative activation, induced by interleukin 4, characterized increased endocytosis, reduced secretion of pro-inflammatory cytokines, roles in immunoregulation remodeling. Although these represent opposite extremes observed vitro , the remarkable plasticity macrophages allows for a wide spectrum phenotypes that are complex characterize experimentally. While application omics techniques has resulted significant advances characterization macrophage polarization, lipidomic studies have received lesser attention. Beyond their role as structural components energy sources, lipids function signaling molecules regulate thereby shaping responses. This work reviews interaction between lipid exploring how metabolism influences phenotype function. These insights offer potential therapeutic strategies immune-mediated diseases inflammation-related disorders, including inflammaging.

Язык: Английский

Процитировано

0

The Interplay Between Immunity, Inflammation and Endothelial Dysfunction DOI Open Access
Ying Jie Chee, Rinkoo Dalan, Christine Cheung

и другие.

International Journal of Molecular Sciences, Год журнала: 2025, Номер 26(4), С. 1708 - 1708

Опубликована: Фев. 17, 2025

The endothelium is pivotal in multiple physiological processes, such as maintaining vascular homeostasis, metabolism, platelet function, and oxidative stress. Emerging evidence the past decade highlighted immunomodulatory function of endothelium, serving a link between innate, adaptive immunity inflammation. This review examines regulation immune-inflammatory axis by discusses immune functions, explores pathophysiological processes leading to endothelial dysfunction various metabolic disturbances, including hyperglycemia, obesity, hypertension, dyslipidaemia. final section focuses on novel, repurposed, emerging therapeutic targets that address dysfunction.

Язык: Английский

Процитировано

0

Changes in feeding behavior, milk yield, serum indexes, and metabolites of dairy cows in three weeks postpartum DOI Creative Commons
Na Liu, Jingwei Qi, Xiaoping An

и другие.

Scientific Reports, Год журнала: 2025, Номер 15(1)

Опубликована: Март 7, 2025

Parturition and lactation stress greatly affect physiological metabolic status of postpartum cows. Monitoring feeding behavior can help assess the health This study aimed to explore changes in behavior, milk yield (MY), serum indexes, metabolites dairy cows during three weeks postpartum. Furthermore, relationships between were investigated. One hundred seventy-eight healthy multiparous Holstein enrolled continuously record variables, including time (FT), frequency (FF), average (AFT), using Nedap neck collar devices, wherein was recorded. Out 178 cows, 20 with same parity number (2 parities), body condition score (3.0 ± 0.25, recorded 7 days before parturition), eutocic calvings, selected indexes on week 1, 2, 3 Then, further based similar calving date (± d) for transition detection. Compared 1 postpartum, FT AFT normalized values 2 significantly increased (P < 0.001). The positively correlated MY 0.01). total protein (TP), cholesterol (T-Chol) IgA contents compared while β-hydroxybutyric acid (BHBA) concentration decreased. Serum malondialdehyde (MDA), interleukin-6 (IL-6), leptin (LEP) 2nd declined 1st negatively BHBA level, TP T-Chol 0.05). According UPLC-MS/MS pathway analysis results, levels glycerophospholipids changed most first which mainly related glycerophospholipid metabolism. Finally, correlation showed that 29 (GPs) fatty acyls (FAs) Together, findings suggest such as AFT, could serve reliable indicators energy metabolism

Язык: Английский

Процитировано

0

Characterization and Comparison of Lipids in Yak Colostrum, Buffalo Colostrum, and Cow Colostrum Based on UHPLC-QTOF-MS Lipidomics DOI Creative Commons
Ruhong Li,

Yuzhuo Wang,

Changhui Li

и другие.

Dairy, Год журнала: 2025, Номер 6(2), С. 14 - 14

Опубликована: Март 27, 2025

Colostrum is a nutrient-dense food rich in proteins, immune modulators, and growth factors essential for neonatal development. Its lipids serve as key energy source facilitate cellular functions. While yak colostrum (YC) buffalo (BC) exhibit high nutritional value, their lipid compositions remain unclear. This study employed lipidomics to analyze compare the profiles of YC BC with cow (CC), identifying 872 across 33 subclasses. Differential analysis revealed 137, 100, 131 lipids, significant expression differences between vs. CC, BC, respectively. exhibited higher levels α-linolenic acid docosahexaenoic acid, suggesting potential role enhancing neurodevelopment cognitive function. In contrast, upregulation specific components such phosphatidylethanolamine (PE) phosphatidylinositol (PI), coupled downregulation lysophosphatidylcholine (LPC) lysophosphatidylethanolamine (LPE), indicated benefit metabolism inflammatory regulation. These findings suggest that may be particularly suitable neonates requiring enhanced support, while offer advantages modulation. The provides critical insights into distinct laying scientific foundation development tailored supplements. results also hold implications dairy industry, driving innovation optimization colostrum-based products meet diverse demands.

Язык: Английский

Процитировано

0

Advancing the Biochemical Understanding of Maple Syrup Urine Disease and the Impact of Liver Transplantation: A Pilot Study DOI
Sotirios P. Fortis, Abby Grier, Julie A. Reisz

и другие.

Journal of Proteome Research, Год журнала: 2025, Номер unknown

Опубликована: Апрель 15, 2025

Maple syrup urine disease (MSUD) is a rare autosomal recessive metabolic disorder causing impaired branched-chain amino acid (BCAA) catabolism and systemic dysregulation. MSUD has an incidence of approximately 1 in 185,000 U.S. births, with much higher prevalence the Mennonite communities (up to 400 live births due c.1312T > A p.Tyr438Asn BCKDHA founder mutation). Using multiomic approach integrating metabolomics, lipidomics, proteomics, we analyzed blood samples from three patients on BCAA-restricted diet (MSUDDR), two who received liver transplants (MSUDLT), six healthy controls. Gene ontology analysis integrated omics data confirmed imbalances MSUD, highlighting increases glycolysis, oxidative phosphorylation, purine metabolism. Lipidomic revealed disruptions sphingolipids lysophosphatidylcholines, affecting signaling membrane integrity. Liver transplantation corrected some abnormalities, but key metabolites proteins remained altered. Proteomic significant alterations redox homeostasis, energy metabolism, cytoskeletal organization partial recovery post-transplantation. Post-translational modifications indicated ongoing stress immune activation MSUDLT group. Elevated levels l-isoleucine, l-valine, their ketoacids persisted post-transplant, correlating lipid remodeling, protein folding. These findings provide insights into MSUD-associated dysfunction highlight potential therapeutic targets.

Язык: Английский

Процитировано

0