Description
Antibody Name: | Anti-NPHP1 Antibody (CAB21079) |
Antibody SKU: | CAB21079 |
Antibody Size: | 50µL, 100µL |
Application: | Western blotting |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 463-732 of human NPHP1 (NP_997064.2). |
Application: | Western blotting |
Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | Mouse testis, Mouse spleen, Rat testis, A-431, 293T, PC-3 |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 463-732 of human NPHP1 (NP_997064.2). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | PDLG ILFE LGIS YIRN STGE RGEL SCGW VFLK LFDA SGVP IPAK TYEL FLNG GTPY EKGI EVDP SISR RAHG SVFY QIMT MRRQ PQLL VKLR SLNR RSRN VLSL LPET LIGN MCSI HLLI FYRQ ILGD VLLK DRMS LQST DLIS HPML ATFP MLLE QPDV MDAL RSSW AGKE STLK RSEK RDKE FLKS TFLL VYHD CVLP LLHS TRLP PFRW AEEE TETA RWKV ITDF LKQN QENQ GALQ ALLS PDGV HEPF DLSE QTYD FLGE MRKN AV |
Cellular Location: | Cell junction, Cell projection, Cytoplasm, adherens junction, cilium, cilium axoneme, cytoskeleton, tight junction |
Calculated MW: | 69kDa/77kDa/83kDa |
Observed MW: | 83kDa |
Synonyms: | NPHP1, JBTS4, NPH1, SLSN1 |
Background: | 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. |
Western blot analysis of extracts of various cell lines, using NPHP1 antibody at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 180s. | |
Western blot analysis of extracts of various cell lines, using NPHP1 antibody at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 90s. |