Polyclonal Rabbit ATP7B Antibody
- Known as:
- Polyclonal Rabbit ATP7B Antibody
- Catalog number:
- abx32832
- Product Quantity:
- EUR
- Category:
- -
- Supplier:
- Abbexa
- Gene target:
- Polyclonal Rabbit ATP7B Antibody
Related genes to: Polyclonal Rabbit 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: Polyclonal Rabbit ATP7B Antibody
Related articles to: Polyclonal Rabbit ATP7B Antibody
- Serotonin transporter Slc6a4a functions as a transporter in serotonin reuptake and is tightly linked with serotonergic regulation and stress responses. However, few studies have investigated its role in copper homeostasis and organogenesis in an in vivo vertebrate model. In this study, we demonstrate that deficiency () leads to copper accumulation, retinal developmental defects, and locomotor dysfunction in zebrafish specifically. Mechanistically, deficiency is associated with reduced and copper accumulation, which lead to reactive oxygen species (ROS) and endoplasmic reticulum (ER) stress, and results in Caspase-3-mediated apoptosis and retinal degeneration. Specifically, tetrathiomolybdate (TTM), a pharmacological copper chelator, partially reduces ER stress and restores retinal defects. Additionally, ectopic expression of full-length mRNA partially restores retinal defects. These findings identify serotonin transporter Slc6a4a as a novel regulator in copper homeostasis and retinal development via the regulation of Atp7b in an in vivo vertebrate model. This study supports a mechanistic link between deficiency, copper overload, and retinal defects and highlights copper chelation as an alternative therapeutic strategy in individuals with deficiency. - Source: PubMed
Publication date: 2026/07/02
Baloch Hameed UllahJing Yuan-YuanShi Jia-HaoWang Han-FeiWu YouLiu Jing-Xia - Genotype-phenotype correlations in Wilson disease (WD) have so far been inconclusive. - Source: PubMed
Publication date: 2026/07/13
Mishra Amresh KumarSen Sarma MoinakDubey AnchalGadekar PratikMoirangthem AmitaSrivastava AnshuMathias Amrita - Wilson disease is an autosomal recessive disorder that affects copper metabolism due to mutations in the ATP7B gene. It causes problems with the liver and the nervous system. On magnetic resonance imaging (MRI), the typical sign is the "face of the giant panda" in the midbrain. A rarer sign, called the "panda with bright eyes," suggests more extensive brainstem involvement and is not often seen. A 21-year-old man developed worsening behavior changes, mood swings, and trouble with his studies. He later had tremors in both arms and mild speech difficulties. His liver tests were abnormal, and his ceruloplasmin level was low at 8 mg/dL. An eye exam showed Kayser-Fleischer rings in both eyes. Brain MRI showed symmetrical T2/FLAIR hyperintensities in the caudate nuclei, putamina, globus pallidi, thalami, posterior limbs of the internal capsules, and both the superior and middle cerebellar peduncles, as well as the pons. Increased T2 signal in the red nuclei and substantia nigra produced the "panda with bright eyes" sign. There was also diffusion restriction in both globus pallidi and thalami. The Leipzig score confirmed Wilson disease. The patient started D-penicillamine treatment and showed clinical improvement. Radiologists need to recognize both common and rare MRI features of Wilson disease, especially when neuropsychiatric symptoms are present. Spotting the "panda with bright eyes" sign can help with early diagnosis, guide metabolic testing, and allow prompt chelation therapy, which can improve neurological outcomes. - Source: PubMed
Publication date: 2026/07/07
Srinivasan SrinidhiPaul ArunSahitya JampanaMulupuru SwathiSakinala Eswar K - Wilson disease (WD) is an autosomal recessive disorder of copper metabolism caused by mutations, leading to pathological copper deposition in the liver, brain, and cornea. Although the gut-liver-brain axis plays a role, direct copper accumulation in multiple organs remains the primary cause of tissue damage. Recent years have seen growing attention to the gut microbiota in WD pathogenesis. Copper imbalance remodels gut microbiota composition and function, while dysbiosis, in turn, affects copper absorption and excretion, forming a vicious cycle that exacerbates multi-organ damage. Copper-induced intestinal barrier disruption, lipopolysaccharide translocation, and systemic inflammation are key links connecting local copper accumulation to systemic injury. This review summarizes the genetic basis of WD, mechanisms of copper toxicity, gut microbiota alterations, and their roles in liver injury and neurodegeneration. It highlights microbiota-derived metabolites-short-chain fatty acids, tryptophan metabolites, bile acids, sulfur-containing amino acids, and branched-chain amino acids-in inter-organ communication. The bidirectional interaction between WD therapies (chelators, zinc salts, dietary interventions) and the gut microbiota is analyzed, along with microbiota-based personalized therapies. However, most current evidence derives from animal models or small cross-sectional studies; large-scale longitudinal human data are critically lacking. A deeper understanding of the gut-liver-brain axis in WD may reveal novel biomarkers and therapeutic targets. - Source: PubMed
Publication date: 2026/06/23
Wang Xue-QiaoWang Yan-XinYe Zhao-HaoZhang Zhi-LinCen Si-Fan - Wilson disease (WD) has long been framed as a hepatocentric disorder of copper accumulation. That view is now giving way to a broader model centered on the gut-liver-kidney-brain axis. In WD, copper is not simply stored in tissues as an inert burden. It circulates in dynamic, bioactive pools-particularly relative exchangeable copper (REC)-that disrupt barrier structures, including the intestinal epithelium and blood-brain barrier, and spread toxicity through measurable biochemical mediators. Major pathogenic processes include copper-induced suppression of autophagy, disruption of FXR-regulated bile acid signaling, and direct injury to the intestinal barrier. Gut dysbiosis, supported by fecal microbiota transplantation (FMT) studies in ATP7B-deficient mice, further amplifies hepatic inflammation and favors copper retention. Renal tubular dysfunction and neurotoxicity appear to reflect copper species-dependent passage across biological barriers together with secondary metabolic disturbances, including the recently described pathway of cuproptosis. In the clinic, this shift has been accompanied by greater use of copper-species biomarkers such as ceruloplasmin oxidase activity and REC, along with advanced imaging approaches such as Cu-PET/CT. Treatment is also moving beyond conventional chelation alone, with increasing attention to biliary copper excretion, epithelial barrier repair, and microbiome-directed interventions. Viewed in this way, the axis model helps explain the marked phenotypic heterogeneity of WD and offers a mechanistic basis for more precise interventions aimed at breaking pathogenic feedback loops across organs. - Source: PubMed
Publication date: 2026/06/17
Qian NannanZhu SihuanSong YuqiYang YulongWang HanHan HuiXu GuocunHao WenjieJiang HailinYang YueXi HuDing YufengHe WeiWei TaohuaYang WenmingCheng Ting