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NPHP1 antibody - middle region (ARP56084_P050)

Description of Target:
Together with Cas NPHP1 may play a role in the control of epithelial cell polarity. NPHP1 seems to help to recruit protein tyrosine kinase 2 beta (PTK2B) to cell matrix adhesions, thereby initiating phosphorylation of PTK2B and PTK2B-dependent signaling.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.
Gene Symbol:
NPHP1
Official Gene Full Name:
Nephronophthisis 1 (juvenile)
NCBI Gene Id:
4867
Alias Symbols:
JBTS4; NPH1; SLSN1
Tissue Tool:
Find tissues and cell lines supported to express NPHP1.
Protein Accession #:
NP_000263
Nucleotide Accession#:
NM_000272
Swissprot Id:
O15259-4
Host:
Rabbit
Clonality:
Polyclonal
Protein Name:
Nephrocystin-1
Protein Size (# AA):
733
Molecular Weight:
83kDa
Application:
WB
Partner Proteins:
ATXN1, PEPD
Immunogen:
The immunogen for anti-NPHP1 antibody: synthetic peptide directed towards the middle region of human NPHP1
Product Format:
Lyophilized powder
Purification:
Affinity Purified
Complete computational species homology data:
NPHP1 antibody - middle region (ARP56084_P050)
Predicted Homology Based on Immunogen Sequence:
Dog: 100%; Pig: 100%; Rat: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Bovine: 100%; Rabbit: 100%; Guinea pig: 93%; Zebrafish: 92%
Species Reactivity:
Mouse, Bovine, Human, Rat, Dog, Pig, Horse, Rabbit, Guinea pig, Zebrafish
Datasheets / Downloads:
Printable datasheet for
anti-NPHP1 antibody
- ARP56084_P050
Peptide Sequence:
Synthetic peptide located within the following region: GILFELGISYIRNSTGERGELSCGWVFLKLFDASGVPIPAKTYELFLNGG
Blocking Peptide:
For anti-NPHP1 antibody is Catalog # AAP56084 (Previous Catalog # AAPP37707)
Key Reference:
Eley,L., (2008) Biochem. Biophys. Res. Commun. 371 (4), 877-882
Reconstitution and Storage:
Add 50 ul of distilled water. Final anti-NPHP1 antibody concentration is 1 mg/ml in PBS buffer with 2% sucrose. For longer periods of storage, store at -20C. Avoid repeat freeze-thaw cycles.
 
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