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
- 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 - Traumatic brain injury (TBI) is a leading cause of disability and mortality, with secondary injury mechanisms involving neuronal death and neuroinflammation, for which effective treatments remain limited. Neutrophil extracellular traps (NETs) are implicated in post-TBI neuropathology. Cuproptosis, a copper-dependent cell death pathway characterized by mitochondrial oxidative stress, dysfunction, and disrupted dynamics, has recently been implicated in neurological disorders. This study aims to investigate whether NETs exacerbate secondary brain injury by promoting neuronal cuproptosis after TBI and to elucidate the underlying molecular mechanism. We observed elevated NET levels in brain tissues from both TBI patients and mice, which correlated with poor prognosis. Single-cell RNA sequencing revealed a significant upregulation of transthyretin (Ttr) in neurons post-TBI. Mechanistically, NETs deliver lactylated S100a9 (S100a9K26la), a glycolysis-dependent lactylated protein, to neurons. S100a9K26la translocates to the nucleus and promotes Ttr transcription. Increased neuronal Ttr protein then competes with ATPase copper transporting β (Atp7b) for binding to copper metabolism MURR1 domain-containing 1 (Commd1) at the W123 residue. This competition disrupts the Commd1-Atp7b interaction, impairing copper efflux and leading to intracellular copper accumulation, mitochondrial oxidative stress, aggregation of DLAT, loss of Fe-S cluster proteins, and ultimately neuronal cuproptosis. Neuron-specific Ttr conditional knockout ameliorated neuronal death, neuroinflammation, blood-brain barrier (BBB) disruption, and neurological deficits in a TBI model. Conversely, inhibition of cuproptosis with the copper chelator tetrathiomolybdate (TTM) yielded similar protective effects. In summary, our findings elucidate a novel pathway wherein NETs, via delivery of S100a9K26la, drive neuronal Ttr overexpression. Ttr disrupts copper homeostasis by interfering with the Commd1-Atp7b axis, ultimately triggering neuronal cuproptosis and exacerbating secondary injury after TBI. This study identifies NETosis and the Ttr/Commd1/Atp7b axis as potential therapeutic targets for mitigating TBI-induced damage. - Source: PubMed
Publication date: 2026/09/21
Xu JianyeZhang XuLiu YangChen BoZhang YaoWang JinchaoCao YiyaoLi ShenghuiWu RuojieSun DongdongLi LeiLiu XiaoYang GuiliWang ZengguangGuo XingLi WeiguoZhang Shu - - Source: PubMed
Publication date: 2026/09/18
Berenguer MarinaTorra MercèEspinós CarmenAndreu Escrivá RocíoPocurull AnnaCarvalho-Gomes ÂngelaMiralpeix AnnaRojas Echarry GustavoRomero-Moreno SaraiSanchez ClaraSilgo EvaPalacios-Martínez ChristianBadenas CeliaPerez-Rojas JudithAguado Codina CristinaMariño Zoe - Cisplatin (CDDP) resistance constitutes the principal clinical challenge in the treatment of advanced non-small cell lung cancers (NSCLC). Disturbances in copper metabolism are closely linked with tumor drug resistance. SOX2, a pivotal stemness transcription factor (TF), holds an important role in chemotherapy resistance. However, whether it influences CDDP resistance by modulating cuproptosis remains unknown. SOX2 level was tested by qPCR and WB, and ATP7B level was evaluated by WB and flow cytometry. The oligomerization levels of FDX1, LIAS, and DLAT, together with Cu levels, were determined by WB, confocal microscopy, and a Cu colorimetric assay kit. The proliferation and apoptosis of NSCLC drug-resistant cells were assessed with CCK-8 and flow cytometry. SOX2 was significantly up-regulated in CDDP-resistant NSCLC cells, accompanied by decreased cuproptosis sensitivity. Silencing SOX2 in CDDP-resistant cells increased cuproptosis sensitivity and apoptosis rate and reduced cell viability. These effects of SOX2 silencing on CDDP resistance in NSCLC cells were reversed by the cuproptosis inhibitor TTM. SOX2 up-regulated ATP7B by activating the Wnt/β-catenin signaling pathway. Silencing ATP7B or treatment with the Wnt pathway inhibitor LF3 attenuated SOX2 overexpression-induced promoting effect on CDDP resistance and suppressive effect on cuproptosis in NSCLC. In summary, this study reveals a novel mechanism wherein SOX2 activates the Wnt/β-catenin signaling pathway to upregulate ATP7B expression, thereby suppressing cuproptosis and ultimately driving cisplatin resistance in NSCLC. These findings provide potential therapeutic targets for reversing chemoresistance in NSCLC. - Source: PubMed
Publication date: 2026/09/18
Chen KunHe Fu-MeiHuang Ying-HuiFang TingZheng Yi-PingCai Jian-Feng