{"product_id":"abcam-ab204951","title":"Abcam, ab204951, Anti-PKHD1 antibody [8G12A1]","description":"\u003cp\u003eSize: 100µg\u003cbr\u003e\nRat Monoclonal PKHD1 antibody. Suitable for Flow Cyt, IHC-P, ICC\/IF and reacts with Human samples. Cited in 4 publications.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rat,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:8G12A1,\u003cbr\u003e\nIsotype:IgG1,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Human,\u003cbr\u003e\nApplications:IHC-P, ICC\/IF, Flow CytSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nThis product was changed from ascites to supernatant. Lot no's high than GR291788-5 are from Tissue Culture Supernatant\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein G, Purification notes-Purified from tissue culture supernatant., Storage buffer-Preservative: 0.05% Sodium azideConstituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze \/ thaw cycle\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nThe PKHD1 gene encodes the polycystic kidney and hepatic disease 1 protein also referred to as fibrocystin\/polyductin. This protein has a molecular mass of approximately 500 kDa. PKHD1 predominantly expresses in kidney and liver epithelial cells. In these tissues it localizes to the primary cilia and the plasma membrane. The structure of PKHD1 suggests it functions as a receptor or co-receptor involved in signal transduction.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nThe PKHD1 protein plays a vital role in maintaining tubular and ductal architecture within the kidney and liver. It likely forms part of a complex allowing proper cellular communication and structural support. PKHD1 interacts with the cellular cytoskeleton suggesting a role in mechanosensation and cell proliferation. These functions contribute to normal tissue development and homeostasis in organs where it is expressed.\u003cbr\u003e\nPathways\u003cbr\u003e\nPKHD1 plays a significant role in the Wnt signaling pathway and ciliary function. These pathways are important for regulating cell division polarity and organization. Within the Wnt signaling pathway PKHD1 interacts with proteins such as β-catenin influencing their stability and translocation. PKHD1's function in cilia suggests a link to cellular processes that depend on precise signal reception and transduction.\u003cbr\u003e\nMutations in PKHD1 are related to autosomal recessive polycystic kidney disease (ARPKD) and congenital hepatic fibrosis. These conditions involve the disrupted function of the kidneys and liver characterized by cyst formation and fibrosis. PKHD1's interaction with fibrocystin\/polyductin is key as defects in this pathway lead to the abnormalities observed in ARPKD. Another protein fibrocystin shares overlapping functions with PKHD1 in these pathological conditions further emphasizing its critical role in maintaining proper kidney and liver function.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46854614483113,"sku":"ab204951","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/es\/products\/abcam-ab204951","provider":"Iright","version":"1.0","type":"link"}