The Anti-NPHP1 Antibody (CAB21079) is a valuable tool for researchers studying the NPHP1 protein, which is involved in the function of primary cilia and ciliopathies. This polyclonal antibody, produced in rabbits, demonstrates high reactivity with human samples and has been validated for use in Western blot applications.NPHP1, also known as nephrocystin-1, is a key player in maintaining the structure and function of primary cilia, which play crucial roles in various cellular processes including signal transduction and cell cycle regulation. Mutations in the NPHP1 gene are associated with a group of genetic disorders known as nephronophthisis, which are characterized by kidney abnormalities and vision impairment.
Research on NPHP1 is essential for understanding the molecular mechanisms underlying ciliopathies and developing potential treatments for disorders associated with primary cilia dysfunction. The Anti-NPHP1 Antibody can aid in the detection and analysis of NPHP1 expression in different cell types, making it an essential tool for studies focused on cilia-related diseases and genetic disorders.
Product Name:
NPHP1 Monoclonal Antibody
SKU:
CAB21079
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human,Mouse,Rat
Immunogen:
Recombinant fusion protein containing a sequence corresponding to amino acids 463-732 of human NPHP1 (NP_997064.2).
This gene encodes a protein with src homology domain 3 (SH3) patterns. This protein interacts with Crk-associated substrate, and it appears to function in the control of cell division, as well as in cell-cell and cell-matrix adhesion signaling, likely as part of a multifunctional complex localized in actin- and microtubule-based structures. Mutations in this gene cause familial juvenile nephronophthisis type 1, a kidney disorder involving both tubules and glomeruli. Defects in this gene are also associated with Senior-Loken syndrome type 1, also referred to as juvenile nephronophthisis with Leber amaurosis, which is characterized by kidney and eye disease, and with Joubert syndrome type 4, which is characterized by cerebellar ataxia, oculomotor apraxia, psychomotor delay and neonatal breathing abnormalities, sometimes including retinal dystrophy and renal disease. Multiple transcript variants encoding different isoforms have been found for this gene.
Purification Method:
Affinity purification
Gene ID:
4867
Clone Number:
ARC52339
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.05% proclin300,0.05% BSA,50% glycerol,pH7.3.
Western blot analysis of various lysates, using NPHP1 Rabbit mAb (CAB21079) at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at1:10000 dilution.Lysates/proteins: 25μg per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit (AbGn00020).Negative control (NC): Mouse spleenExposure time: 90s.
