Anti-Fibrocystin Antibody, clone 18

产品说明

一般描述

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.

特异性

Expected to react with both spliced isoforms.

免疫原

Epitope: N-terminal half
Recombinant protein corresponding to human Fibrocystin fragment from the N-terminal half.

应用

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).
This Anti-Fibrocystin Antibody, clone 18 is validated for use in Immunohistochemistry (Paraffin), Immunocytochemistry for the detection of Fibrocystin.
Research Category
Signaling
Research Sub Category
Signaling Neuroscience

质量

Evaluated by Immunohistochemistry in human kidney medulla tissue.

Immunohistochemistry Analysis: A 1:50 dilution of this antibody detected Fibrocystin in human kidney medulla tissue.

目标描述

~371 to 446 kDa calculated

外形

Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Format: Purified
Protein G Purified

储存及稳定性

Stable for 1 year at 2-8°C from date of receipt.

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

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.

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