epitope corresponding to amino acids 433-732 mapping at the C-terminus of nephrocystin of human origin
Modification Notes
Clinical features of familial juvenile nephronophthisis include anemia, polyuria, polydipsia, isosthenuria, and death in uremia (1). Juvenile nephronophthisis type 1 is caused by mutations of NPHP1, the gene encoding for nephrocystin (1,2). Nephrocystin interacts with p130Cas (BCAR1), proline-rich tyrosine kinase-2 (PTK2B or Pyk2), and tensin in embryonic kidney and testis, indicating that these proteins participate in a common signaling pathway (1,2). Nephrocystin and p130Cas interact in mammalian cells and both proteins prominently localize at or near sites of cell-cell contact in polarized Madin-Darby canine kidney epithelial cells (3). Expression of nephrocystin results in phosphorylation of Pyk2 on Tyrosine 402 as well as activation of downstream mitogen-activated protein kinases, such as ERK1 and ERK2 (1,2). Nephrocystin contains a src-homology 3 (SH3) domain, which is highly conserved throughout evolution (4). The gene which encodes nephrocystin maps to human chromosome 2q13 (5).
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WBIPIFELISA
recommended for detection of nephrocystin isoforms 1-3 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine:
Species Reactivity
HumanMouseRat
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Alternative Names
epitope corresponding to amino acids 433-732 mapping at the C-terminus of nephrocystin of human origin
Tissue Specificity
epitope corresponding to amino acids 433-732 mapping at the C-terminus of nephrocystin of human origin
Western blot analysis of nephrocystin expression in non-transfected: (A) and human nephrocystin transfected: (B) 293T whole cell lysates.
Application Notes
recommended for detection of nephrocystin isoforms 1-3 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine:
Additional Information
Form
Liquid
Storage Instructions
For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.