ATP7b ELISA kit
- Known as:
- ATP7b Enzyme-linked immunosorbent assay test reagent
- Catalog number:
- DL-ATP7b-Mu
- Product Quantity:
- 96T
- Category:
- Elisa Kits
- Supplier:
- WDSTD
- Gene target:
- ATP7b ELISA kit
Related genes to: ATP7b ELISA kit
- 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 ELISA kit
Human ELC ELISA KIT 96 TEST
OxiSelect In Vitro ROS/RNS Assay Kit (Green Fluorescence), Trial Size
OxiSelect Methylglyoxal (MG) Competitive ELISA Kit
OxiSelect Methylglyoxal (MG) Competitive ELISA Kit
OxiSelect TBARS Assay Kit (MDA Quantitation), Trial Size
OxiSelect Total Antioxidant Capacity (TAC) Assay Kit, Trial Size
OxiSelect™ In Vitro ROS RNS Assay Kit (Green Fluorescence), Trial Size(1-3)-beta-D-glucan Sandwich ELISA, Double Antibody(1-Kit )11,12-EET DHET Immunoassay Kit(1-Kit )11,12-EET_DHET Immunoassay Kit(1-Kit) 11,12-DHET Immunoassay Kit(1-Kit) 14,15-DHET Human Urine ELISA Kit(1-Kit) 14,15-DHET Hypertension ELISA Kit(1-Kit) 14,15-DHET sEH activity ELISA Kit(1-Kit) 14,15-EET DHET Hypertension ELISA Kit Related articles to: ATP7b ELISA kit
- Copper (Cu) transporting ATPases represent a highly conserved subclass of P-type ATPases with critical roles in Cu export and metalloenzyme synthesis. Despite their important biological roles and association with a wide range of human diseases, no high-affinity small-molecule inhibitors have been described. Here, we identify MKV3 as a small molecule inhibitor of Cu-transporting P-type ATPases that targets a conserved Cu entry site to the translocation pathway. In silico docking against the ATP7B structure revealed a highly conserved pocket suitable for pharmacological inhibition. MKV3 bound human ATP7A and ATP7B with nanomolar affinity, competed with N-terminal metal-binding domains for access to the Cu entry site, and selectively inhibited CopA ATPase activity and Cu transport. Mechanistically, MKV3 blocked chaperone-mediated Cu delivery to the intramembranous CPC site of CopA that is essential for its transport function. We further identified a single charged P-domain residue that governed MKV3 affinity and potency across species. Functionally, MKV3 phenocopied the genetic loss of Cu-ATPases in bacteria, fungi, plants, zebrafish, and mammals, impairing copper-dependent enzymes, transporter trafficking, and copper tolerance. These findings establish a conserved, druggable vulnerability in Cu-ATPases and introduce MKV3 as a broadly active chemical tool to modulate copper homeostasis across biological kingdoms. - Source: PubMed
Publication date: 2026/05/14
Shanbhag Vinit CAnakpeba-Dinguyella SamuelGudekar NikitaConrad KristynAzubuogu ChiemerieProbst CorinnaRalle MartinaMediavilla María GCricco Julia AGarza Natalie MGohil Vishal MPeck ScottKumar SiddharthaNatarajan AmarnathHoradigala-Gamage Madujika AMeloni GabrieleSingh KamalPetris Michael J - Wilson disease (WD) is a hereditary autosomal recessive disorder of copper metabolism, which results from mutations in the ATP7B gene. It is a monogenic disorder characterized by significant clinical heterogeneity in patients with renal, ocular, hepatic, and neurological involvement, complicating its clinical diagnosis. This study involves examination of an Iranian family with a child diagnosed with WD. - Source: PubMed
Publication date: 2026/05/13
Hasani ElahehKohkalani MoeinNaghinejad MaryamDerakhshan Sima MansooriKhaniani Mahmoud ShekariTaheri Mohammad - Wilson's disease (WD) is an autosomal recessive disorder of copper metabolism caused by mutations, characterized by hepatic copper accumulation and multisystem involvement. Several rare inherited and acquired conditions can closely mimic WD, posing diagnostic challenges and the risk of inappropriate therapy. By examining neuroimaging patterns and distinguishing between diagnostic criteria, this narrative review provides a comprehensive synthesis of WD-mimicking disorders, emphasizing their molecular mechanisms, clinical phenotypes, and biochemical features. WD-mimicking disorders encompass -related neurodegenerations (Menkes disease, occipital horn syndrome, X-linked distal hereditary motor neuropathy), MEDNIK syndrome, Huppke-Brendel syndrome, aceruloplasminemia, congenital disorders of glycosylation, primary familial intrahepatic cholestasis type 3, and acquired copper deficiency syndromes. Mechanisms include systemic copper deficiency, impaired intracellular trafficking, defective ceruloplasmin biosynthesis, secondary hepatic copper accumulation, and abnormal glycosylation. Clinical features range from neurodevelopmental delay, movement disorders, and hepatic dysfunction to dermatologic, hematologic, and connective-tissue abnormalities. Biochemical profiles may overlap with WD, particularly low serum ceruloplasmin and total copper, altered urinary copper excretion, and elevated hepatic copper in some disorders. Neuroimaging and genetic testing provide critical discriminative value. Management is largely supportive, with disease-specific therapies available in selected conditions, such as subcutaneous copper in Menkes disease or monosaccharide supplementation in certain congenital disorders of glycosylation subtypes. Accurate differentiation between WD and WD-mimicking disorders requires careful integration of clinical, biochemical, imaging, and molecular data. Recognition of distinctive features and understanding underlying pathophysiology are essential to avoid misdiagnosis and inappropriate anti-copper therapy, optimize management, and improve patient outcomes. - Source: PubMed
Publication date: 2026/04/29
Antos AgnieszkaGromadzka GrażynaBembenek Jan PawełLitwin Tomasz - Diagnostic delay in Wilson's disease (WD) remains common because of marked phenotypic heterogeneity. Although neuropsychiatric presentations are known to delay recognition, quantitative evidence linking delayed diagnosis to transplant-free survival is limited, particularly in mixed hepatic-neurologic phenotypes. We aimed to characterize diagnostic delay and misdiagnosis, identify associated phenotypes, and evaluate the independent association of delayed diagnosis with transplant-free survival. - Source: PubMed
Publication date: 2026/04/02
Özden YavuzSağlam Osman - Wilson's disease (WD) is an autosomal recessive disorder caused by ATP7B mutations, resulting in impaired copper metabolism and progressive neuropsychiatric manifestations. This study investigated spatiotemporal alterations in regional brain activity using static and dynamic resting-state fMRI with regional homogeneity (ReHo), and their relationships with clinical features. - Source: PubMed
Publication date: 2026/04/20
Zhou ZhihuaXiao WenqingYang NingLin WeizhaoChen ZichaoLiang ManLi JiejingWu Yunfan