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Anti-Fibrocystin Antibody, clone 18

产品编号:4154370
规格:clone 18, from mouse
包装规格:100 μG
产品类别:进口试剂
品牌:Sigma-Aldrich
优惠价:立即咨询
产品价格
产品编号包装单位单价(元)国内现货国外库存询价单
4154370100 μG5350
产品别名

Anti-Fibrocystin Antibody, clone 18

Fibrocystin, Polycystic kidney and hepatic disease 1 protein, Polyductin, Tigmin, Fibrocystin

基本信息
eCl@ss
32160702
General description【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.
Specificity【特异性】
Expected to react with both spliced isoforms.
Immunogen【免疫原】
Epitope: N-terminal half
Recombinant protein corresponding to human Fibrocystin fragment from the N-terminal half.
Application【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
Quality【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.
Physical form【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
Other Notes【其他说明】
Concentration: Please refer to lot specific datasheet.
产品性质
Quality Level【质量水平】
100
biological source【生物来源】
mouse
antibody form【抗体形式】
purified antibody
antibody product type
primary antibodies
clone【克隆】
18, monoclonal
species reactivity
human, rat
technique(s)
immunocytochemistry: suitable
immunohistochemistry: suitable (paraffin)
isotype【同位素/亚型】
IgG2aκ
NCBI accession no.【NCBI登记号】
NP_619639
UniProt accession no.【UniProt登记号】
P08F94
shipped in【运输】
wet ice
Gene Information
human ... PKHD1(5314)
产品说明
Target description【目标描述】
~371 to 446 kDa calculated
Storage and Stability【储存及稳定性】
Stable for 1 year at 2-8°C from date of receipt.
Disclaimer【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.
安全信息
Storage Class Code【储存分类代码】
12 - Non Combustible Liquids
WGK
WGK 1
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