ATP7B antibody
- Known as:
- ATP7B (anti-)
- Catalog number:
- orb10160
- Product Quantity:
- EUR
- Category:
- -
- Supplier:
- Biorbyt biorb
- Gene target:
- ATP7B antibody
Related genes to: ATP7B antibody
- Gene:
- ATP7B NIH gene
- Name:
- ATPase copper transporting beta
- Previous symbol:
- WND
- Synonyms:
- -
- Chromosome:
- 13q14.3
- Locus Type:
- gene with protein product
- Date approved:
- 1986-01-01
- Date modifiied:
- 2016-02-10
Related products to: ATP7B antibody
Related articles to: ATP7B antibody
- Copper is a widespread heavy metal pollutant in aquatic ecosystems, but the systemic molecular mechanisms of copper-induced hepatotoxicity in fish remain incompletely understood. Here, we integrated histopathological examination, biochemical assays, transcriptomics, and metabolomics to investigate the hepatotoxic effects of copper sulfate (CuSO₄) exposure in Oreochromis niloticus. CuSO₄ exposure caused typical pathological damage, including hepatocyte swelling, vacuolar degeneration, and pyknosis. Biochemical assays revealed significantly reduced activities of superoxide dismutase (SOD) and catalase (CAT), decreased glutathione (GSH) content, and elevated malondialdehyde (MDA) levels, indicating severe oxidative stress. Transcriptomic analysis identified 896 differentially expressed genes (DEGs) enriched in pathways related to lipid and fatty acid metabolism, cytochrome P450, and PPAR signaling. Metabolomic analysis detected 738 differentially expressed metabolites (DEMs), primarily involved in glutathione metabolism, oxidative phosphorylation, and amino acid metabolism. Integrated multi-omics analysis uncovered two core mechanisms: depletion of the glutathione system (significantly reduced GSH and GSSG) and energy metabolic reprogramming (marked decreases in NADH, citrate, and acylcarnitines). Concurrently, immune- and inflammation-related genes (IL1B, IL8, C3, C7) were strongly upregulated. Notably, copper homeostasis genes (ATP7B, CP) were downregulated, suggesting that copper accumulation may drive mitochondrial dysfunction and metabolic collapse. In conclusion, CuSO₄ induces hepatotoxicity in tilapia primarily by triggering oxidative stress, which is characterized by glutathione depletion and disruption of energy and lipid metabolic homeostasis. These findings provide multi-omics evidence for understanding copper toxicity and offer potential biomarkers for environmental risk assessment of copper pollution in aquatic ecosystems. - Source: PubMed
Publication date: 2026/09/24
Chen JianjieHan QianxiLiu BoCheng YanfenHuang TingLuo YongjuCao Jinling - Wilson disease is a prototypical disorder of copper metabolism in which hepatic copper overload drives progressive liver injury and fibrosis, yet the molecular mechanisms linking copper accumulation to fibrogenesis remain poorly defined. Here, we identify osteopontin (OPN) as a copper-responsive hepatokine that mechanistically couples copper overload to hepatic stellate cell activation. Using ATP7B-deficient models, we show that hepatic copper accumulation is accompanied by liver injury, inflammatory infiltration, and fibrotic remodeling, together with marked induction of OPN. In hepatocytes, copper exposure led to intracellular copper accumulation and oxidative stress, resulting in robust upregulation and secretion of OPN, which was partially attenuated by antioxidant treatment. Conditioned medium from copper-stressed hepatocytes promoted stellate cell activation and extracellular matrix deposition, whereas genetic depletion of SPP1 in hepatocytes markedly blunted these pro-fibrotic effects, establishing OPN as a key paracrine mediator of copper-induced hepatocyte-stellate cell crosstalk. Clinically, hepatic and circulating OPN levels were significantly elevated in patients with Wilson disease and closely associated with copper burden, fibrosis severity, and liver stiffness. Moreover, serum OPN independently predicted histological fibrosis with good diagnostic performance. Collectively, these findings reveal a copper-driven secretory pathway that links disrupted copper homeostasis to liver fibrogenesis and highlight OPN as a metal-responsive mediator and potential biomarker in Wilson disease. - Source: PubMed
Publication date: 2026/09/26
Li YingjieWu YaoyiWang SujuanZhou Ning - Polystyrene microplastics (PS-MPs) are increasingly recognized for their detrimental effects on reproductive health, particularly in the male reproductive system. While mitochondrial oxidative stress is recognized as a driver of MPs-induced reproductive injury, it remains unclear whether this process involves cuproptosis, a newly identified copper-dependent form of regulated cell death, and elucidating this potential link is critical for alleviating such damage. Here, we report that PS-MPs exposure induces testicular injury and spermatogenic dysfunction in mice, associated with redox imbalance, copper transport defects, and upregulation of the cuproptosis regulators Ferredoxin 1 (FDX1) and Lipoic Acid Synthetase (LIAS). In turn, zinc-doped trifolirhizin-derived carbon dots (ZnTFZCDs) were rationally designed via a one-step hydrothermal strategy, embedding the antioxidant properties of a natural flavonoid and the reproductive-protective role of zinc into a biocompatible carbon nanoplatform. ZnTFZCDs are well-dispersed carbon dots rich in oxygen/nitrogen-containing groups with Zn coordination, and demonstrate favorable biosafety without overt cytotoxicity, hemolysis, or organ damage. , ZnTFZCDs potently scavenge intracellular reactive oxygen species (ROS), maintain antioxidant enzyme activities, preserve mitochondrial membrane potential, suppress lipid peroxidation, and rescue Glutathione peroxidase 4 (GPX4) expression in MPs-exposed RAW264.7 and GC-2 cells. Notably, ZnTFZCDs modulate copper homeostasis by upregulating the copper exporter ATP7B, downregulating the importer SLC31A1, and reducing FDX1/LIAS expression, thereby inhibiting cuproptosis-related signaling. , ZnTFZCDs administration ameliorates testicular histopathology, improves sperm count and morphology, reactivates the Nrf2/HO-1 antioxidant axis, and suppresses cuproptosis-associated alterations in testicular tissues, confirming the translational potential of the findings. Collectively, ZnTFZCDs represent a bio-inspired nanoplatform that attenuates PS-MPs-induced male reproductive injury through coordinated modulation of redox and copper homeostasis, offering a new therapeutic strategy for environmental pollutant-related reproductive disorders by targeting the previously overlooked cuproptosis pathway. - Source: PubMed
Publication date: 2026/09/15
Li TushuaiLi KehuiYang ShiqingPan HongyingCai ZhihuiHua XiyaoLiao GuangfuLuo BingZhang Jie - Decreasing DNA sequencing costs has paved the way to the use of next generation sequencing (NGS) to expand the number of conditions tested in routine newborn screening (NBS) programs. Expanding NBS programs by NGS, however, raises technical, ethical and economic concerns that are important for low-middle income countries or countries that are hesitant to use sequencing in a public health setting. qPCR-based DNA analyses have already been implemented in routine NBS programs; however, qPCR cannot be highly multiplexed. digitalMLPA (dMLPA) is a highly multiplexed probe-based DNA technique. Here, we describe the new dMLPA EZtec-MS technique that was specifically developed for use on dried blot spots (DBS) and can target up to 1200 DNA sequences representing different variant types, allowing high-throughput testing of crude DNA extracts prepared from a single DBS punch. The assay was validated using positive DNA samples and crude DNA extracts from DBS of 2069 negatively screened Philippine newborns. The EZtec-MS assay used in this study successfully detected copy number variants, (including complex genomic regions (SMN1/2, HBA1/2)), inversions (F8), methylation (imprinting diseases, Fragile X), single nucleotide variants (including ATP7B, CTNS, MT-RNR1), and low copy number sequences (TRECs for SCID, cytomegalovirus) in a single reaction in both positive DNA and crude DNA extracts from DBS. The results also demonstrated that dMLPA EZtec-MS is a high-throughput method, suitable for DBS, and has a relatively short turn-around time of 36-40 h. Our findings indicate that the dMLPA EZtec-MS technique is a promising versatile tool for DNA-based NBS programs. - Source: PubMed
Publication date: 2026/09/22
Fabella Terence Dianeden Hoed JoeryCutiongco-de la Paz Eva MariaPadilla CarmencitaHettinga Chrisde Groot KarelLodén-van Straaten MartinSantos Tatiana Cvan den Berg RichielSistermans Erik ASchouten Jan - Copper is an essential micronutrient whose redox activity underpins a dual role in immunity: it serves as both an antimicrobial effector and a regulator of inflammatory signaling. Macrophages, as central orchestrators of innate and adaptive immunity, maintain sophisticated copper homeostasis mechanisms that dynamically adapt to distinct activation states and environmental cues. This review synthesizes current knowledge around three interconnected themes: (1) the molecular machinery governing copper transport and regulation-including CTR1, ATP7A, ATP7B, and copper chaperones; (2) the functional interplay between copper metabolism, macrophage polarization, and immunometabolism; and (3) the pathophysiological consequences of copper dysregulation in infection, chronic inflammation, and cancer. Emerging evidence reveals that copper exerts dose-dependent effects on macrophage polarization: low-to-moderate copper promotes an anti-inflammatory M2 phenotype via STAT6 and PI3K/Akt pathways, whereas high copper concentrations trigger oxidative stress and NF-κB activation, driving pro-inflammatory M1 polarization. Furthermore, recent findings highlight crosstalk among copper metabolism, cuproptosis, and tumor-associated macrophage function, opening new avenues for copper-based immunotherapy. This review identifies critical knowledge gaps-including tissue-specific copper regulation, single-cell dynamics of copper trafficking, and the therapeutic potential of copper-targeted interventions. While acknowledging the bidirectional causality between copper metabolism and macrophage activation, we argue that copper homeostasis functions as a rheostat of immune competence with substantial translational promise. - Source: PubMed
Publication date: 2026/09/07
Yang WantingWang JunfengDong ZhenbinZhang FangmingLi GuofengWang XingXie Wensheng