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Anti-Fibrocystin, clone 18 (mouse monoclonal)

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货号：MABS1156

品牌：Millipore 密理博

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重要规格表

Species Reactivity Key Applications Host Format Antibody Type
H, R IH(P), ICC M Purified Monoclonal Antibody
Description
Catalogue Number MABS1156
Description Anti-Fibrocystin Antibody, clone 18
Alternate Names
  • Fibrocystin
  • Polycystic kidney and hepatic disease 1 protein
  • Polyductin
  • Tigmin
  • Fibrocystin
Background Information Fibrocystin (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.
References
Product Information
Format Purified
Presentation Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Applications
Application This Anti-Fibrocystin Antibody, clone 18 is validated for use in Immunohistochemistry (Paraffin), Immunocytochemistry for the detection of Fibrocystin.
Key Applications
  • Immunohistochemistry (Paraffin)
  • Immunocytochemistry
Application Notes Immunohistochemistry 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
Immunogen Recombinant protein corresponding to human Fibrocystin fragment from the N-terminal half.
Epitope N-terminal half
Clone 18
Concentration Please refer to lot specific datasheet.
Host Mouse
Specificity Expected to react with both spliced isoforms.
Isotype IgG2aκ
Species Reactivity
  • Human
  • Rat
Antibody Type Monoclonal Antibody
Entrez Gene Number
Gene Symbol
  • PKHD1
  • FCYT
  • TIGM1
Purification Method Protein G Purified
UniProt Number
Molecular Weight ~371 to 446 kDa calculated
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality Assurance Evaluated 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 Conditions Stable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size 100 μg
Transport Information
Supplemental Information
Specifications

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