MABS1156

Anti-Fibrocystin Antibody, clone 18

clone 18, from mouse

• 동의어(들): Fibrocystin, Polycystic kidney and hepatic disease 1 protein, Polyductin, Tigmin, Fibrocystin
• UNSPSC 코드: 12352203
• eCl@ss: 32160702
• NACRES: NA.41

속성

• 생물학적 소스: mouse
• Quality Level: 100
• 항체 형태: purified antibody
• 항체 생산 유형: primary antibodies
• 클론: 18, monoclonal
• 종 반응성: human, rat
• 기술: immunocytochemistry: suitable; immunohistochemistry: suitable (paraffin)
• 동형: IgG2aκ
• NCBI 수납 번호: NP_619639
• UniProt 수납 번호: P08F94
• 배송 상태: wet ice
• 타겟 번역 후 변형: unmodified
• 유전자 정보: human ... PKHD1(5314)

설명

일반 설명

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.

특이성

Expected to react with both spliced isoforms.

면역원

Epitope: N-terminal half

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

애플리케이션

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).

Research Category
Signaling

Research Sub Category
Signaling Neuroscience

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

품질

Evaluated by Immunohistochemistry in human kidney medulla tissue.

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

표적 설명

~371 to 446 kDa calculated

물리적 형태

Format: Purified

Protein G Purified

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

저장 및 안정성

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

기타 정보

Concentration: Please refer to lot specific datasheet.

면책조항

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.

안전성 정보

• Storage Class Code: 12 - Non Combustible Liquids
• WGK: WGK 1
• Flash Point (°F): Not applicable
• Flash Point (°C): Not applicable