Polycystic Kidney Disease 1 (Autosomal Dominant) (PKD1) Antikörper

Antigen: Polycystic Kidney Disease 1 (Autosomal Dominant) (PKD1)

Synonyme: PBP, Pc-1, PC1, FLJ00285, MGC118471, mFLJ00285

Klonalität: Polyklonal

Wirt: Kaninchen

Reaktivität: Human

Applikation: Immunhistochemie (IHC), Western Blot (WB)

Produktnummer: ABIN350752

Produktbeschreibung

Weitere Bezeichnung: polycystic kidney disease protein 1 (PKD1, Polycystin-1, PBP) (a PKD domain in 1t extracellular loop)

Swiss-Prot: P98161

Immunogen: A synthetic peptide from a PKD domain in 1t extracellular loop of human PKD1 (Polycystin-1, PBP) conjugated to an immunogenic carrier protein was used as the immunogen.

Format: Lyophilized

Beschreibung: This gene encodes a member of the polycystin protein family. The encoded glycoprotein contains a large N-terminal extracellular region, multipletransmembrane domains and a cytoplasmic C-tail. It may function as an integral membrane protein involved in cell-cell/matrix interactions, and may modulate intracellular calcium homoeostasis and other signal-transduction pathways. It plays a role in renal tubular development, and mutations in this gene have been associated with autosomal dominant polycystic kidney disease. Splice variants encoding different isoforms have been noted for this gene. Also, six pseudogenes, closely linked in a known duplicated region on chromosome 16p, have been described. FUNCTION: May be an ion-channel regulator. PKD1 and PKD2 may function through a common signaling pathway that is necessary for normal tubulogenesis. Involved in adhesive protein-protein and protein-carbohydrate interactions. SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein. Also known as: Polycystin-1, PKD1, Autosomal dominant polycystic kidney disease protein 1, PBP.

Spezifität: Appears to be specific for PKD1.

Anwendungen

Applikationshinweise: IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Reinheit: whole serum

Lagerung: Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Publikationen

Publikationen: Hughes, Ward, Peral et al.: "The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains." in: Nature genetics, Vol. 10, Issue 2, pp. 151-60, 1995 (PubMed).

Peral, Gamble, Strong et al.: "Identification of mutations in the duplicated region of the polycystic kidney disease 1 gene (PKD1) by a novel approach." in: American journal of human genetics, Vol. 60, Issue 6, pp. 1399-410, 1997 (PubMed).

Perrichot, Mercier, Quere et al.: "Novel mutations in the duplicated region of PKD1 gene." in: European journal of human genetics : EJHG, Vol. 8, Issue 5, pp. 353-9, 2000 (PubMed).

Phakdeekitcharoen, Watnick, Ahn et al.: "Thirteen novel mutations of the replicated region of PKD1 in an Asian population." in: Kidney international, Vol. 58, Issue 4, pp. 1400-12, 2000 (PubMed).

Bouba, Koptides, Mean et al.: "Novel PKD1 deletions and missense variants in a cohort of Hellenic polycystic kidney disease families." in: European journal of human genetics : EJHG, Vol. 9, Issue 9, pp. 677-84, 2001 (PubMed).

Tsuchiya, Komeda, Takahashi et al.: "Mutational analysis within the 3' region of the PKD1 gene in Japanese families." in: Mutation research, Vol. 458, Issue 3-4, pp. 77-84, 2001 (PubMed).

Stayner, Zhou: "Polycystin channels and kidney disease." in: Trends in pharmacological sciences, Vol. 22, Issue 11, pp. 543-6, 2001 (PubMed).

Olsen, Blagoev, Gnad et al.: "Global, in vivo, and site-specific phosphorylation dynamics in signaling networks." in: Cell, Vol. 127, Issue 3, pp. 635-48, 2006 (PubMed).