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MABS1156 | Anti-Fibrocystin Antibody, clone 18

100 μg  
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      Replacement Information

      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, R IH(P), ICC M Purified Monoclonal Antibody
      Catalogue NumberMABS1156
      DescriptionAnti-Fibrocystin Antibody, clone 18
      Alternate Names
      • Fibrocystin
      • Polycystic kidney and hepatic disease 1 protein
      • Polyductin
      • Tigmin
      • Fibrocystin
      Background InformationFibrocystin (UniProt P08F94; also known as Polycystic kidney and hepatic disease 1 protein, Polyductin, Tigmin, TIG multiple domains 1, Tigmin) is encoded by the PKHD1 (also known as ARPKD, FCYT, TIGM1) gene (Gene ID 5314) in human. Autosomal recessive polycystic kidney disease (ARPKD) is the most common cause of hereditary childhood PKD. ARPKD is caused by mutations in the gene PKHD1, which encodes fibrocystin/polyductin (FCP), while mutations in two other genes PKD1 and PKD2 that code polycystin-1 (PC1) and polycystin-2 (PC2), respectively, are the cause of autosomal dominant PKD (ADPKD). These three PKD proteins have been localized to renal primary cilia, where the PC1/PC2 complex acts as a flow sensor on the cilium. Fibrocystin is known to complex with PC2, but its biological role is unclear, While Uniprot (P08F94) documents two alternatively spliced human fibrocytin isoforms, multiple lines of evidence indicate that many more spliced mRNA transcripts exist and that aberrant mRNA splicing may represent a pathogenic mechanism in ARPKD in addition to gene mutations.
      Product Information
      PresentationPurified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      ApplicationThis Anti-Fibrocystin Antibody, clone 18 is validated for use in Immunohistochemistry (Paraffin), Immunocytochemistry for the detection of Fibrocystin.
      Key Applications
      • Immunohistochemistry (Paraffin)
      • Immunocytochemistry
      Application NotesImmunohistochemistry Analysis: A 1:50 dilution from a representative lot detected Fibrocystin in human kidney cortex, rat kidney cortex, and rat kidney medulla tissue.
      Immunocytochemistry Analysis: A representative lot detected fibrocystin immunoreactivity associated with primary cilia of cultured normal human kidney epithelial cells (Courtesy of Dr Christopher Ward, University of Kansas Medical Center).
      Biological Information
      ImmunogenRecombinant protein corresponding to human Fibrocystin fragment from the N-terminal half.
      EpitopeN-terminal half
      ConcentrationPlease refer to lot specific datasheet.
      SpecificityExpected to react with both spliced isoforms.
      Species Reactivity
      • Human
      • Rat
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • PKHD1
      • FCYT
      • TIGM1
      Purification MethodProtein G Purified
      UniProt Number
      Molecular Weight~371 to 446 kDa calculated
      Physicochemical Information
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Immunohistochemistry in human kidney medulla tissue.

      Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected Fibrocystin in human kidney medulla tissue.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 μg
      Transport Information
      Supplemental Information




      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-Fibrocystin, clone 18 -Q2595871 Q2595871


      Reference overviewPub Med ID
      Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia.
      Ward, CJ; Yuan, D; Masyuk, TV; Wang, X; Punyashthiti, R; Whelan, S; Bacallao, R; Torra, R; LaRusso, NF; Torres, VE; Harris, PC
      Human molecular genetics 12 2703-10 2003

      Show Abstract
      12925574 12925574


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