C1749
Monoclonal Anti-Polycystin-1 antibody produced in mouse
clone PKD46, tissue culture supernatant, buffered aqueous solution
Synonym(s):
Anti-PCKD, Anti-PKD1, Anti-Polycystic kidney disease 1 (autosomal dominant), Anti-Polycystic kidney disease-associated protein
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About This Item
UNSPSC Code:
12352203
NACRES:
NA.41
Recommended Products
biological source
mouse
conjugate
unconjugated
antibody form
tissue culture supernatant
antibody product type
primary antibodies
clone
PKD46, monoclonal
form
buffered aqueous solution
mol wt
antigen ~500 kDa
species reactivity
human, rat
technique(s)
immunohistochemistry: 1:50-1:100 using human kidney sections
isotype
IgM
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
Gene Information
human ... PKD1(5310)
General description
Monoclonal Anti-Polycystin-1 (mouse IgM isotype) is derived from the hybridoma PKD46 produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice immunized with human polycystin-1, conjugated to KLH. Polycystin-1 is composed of a large N-terminal extracellular region that contains several ligand-binding domains, multiple transmembrane domains and a cytoplasmic C-tail. It has a wide tissue distribution, with higher expression in the kidney, brain, liver, pancreas, heart and intestine.
Polycystin-1 is a protein encoded by the PKD1 gene in humans. It is also referred as PBP, Pc-1 and TRPP1. Polycystic kidney disease 1 (PKD1) gene is found on chromosome 16. It is a glycoprotein with multiple transmembrane domains and a cytoplasmic C-tail.
Immunogen
synthetic peptide corresponding to amino acids 4282-4300 of human polycystin-1, conjugated to KLH.
Application
Monoclonal Anti-Polycystin-1 antibody produced in mouse has been used in immunoblotting and immunohistochemistry.
Biochem/physiol Actions
PKD1 protein is involved in the adhesion of protein-protein and protein-carbohydrate interactions in the extracellular compartment. The most common cause of autosomal dominant polycystic kidney disease (ADPKD) is due to mutation in the PKD1 gene. Alterations in single base substitutions within the coding sequence lead to missense or synonymous mutations in PKD1 gene. ADPKD caused by mutations in PKD1 gene is more severe than that caused by PKD2 gene mutations.
Polycystin-1 is associated with renal tubulogenesis and intracellular signaling pathways.
Physical form
Solution containing 15 mM sodium azide.
Disclaimer
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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Polycystin: new aspects of structure, function, and regulation
Wilson PD
Journal of the American Society of Nephrology, 12(4), 834-845 (2001)
Rodney D Gilbert et al.
Pediatric nephrology (Berlin, Germany), 28(11), 2217-2220 (2013-04-30)
Dominant polycystic kidney disease is common and usually presents clinically in adulthood. Recessive polycystic kidney disease is much less common and frequently presents antenatally or in the neonatal period with severe renal involvement. These are usually thought of as clinically
Jana Reiterová et al.
BMC nephrology, 14, 59-59 (2013-03-19)
Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of inherited kidney disease that results in renal failure. ADPKD is a systemic disorder with cysts and connective tissue abnormalities involving many organs. ADPKD caused by mutations in PKD1
Francisco J Gonzalez-Paredes et al.
Gene, 546(2), 243-249 (2014-06-08)
Autosomal dominant polycystic kidney disease is the most common human monogenic disorder and is caused by mutations in the PKD1 or PKD2 genes. Most patients with the disease present mutations in PKD1, and a considerable number of these alterations are
Cell, 81(2), 289-298 (1995-04-21)
Mutations in the PKD1 gene are the most common cause of autosomal dominant polycystic kidney disease (ADPKD). Other PKD1-like loci on chromosome 16 are approximately 97% identical to PKD1. To determine the authentic PKD1 sequence, we obtained the genomic sequence
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