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Key Specifications Table
| 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 |
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| 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. |
| 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. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | This Anti-Fibrocystin Antibody, clone 18 is validated for use in Immunohistochemistry (Paraffin), Immunocytochemistry for the detection of Fibrocystin. |
| Key Applications |
|
| 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 |
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| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| Gene Symbol |
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| Purification Method | Protein G Purified |
| UniProt Number |
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| Molecular Weight | ~371 to 446 kDa calculated |
| 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 |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 μg |