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Nephrocystin 1 (NPHP1) Antibody
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Catalogue No: abx005117
Price:
US$203.00
(Size: 20 µl)
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NPHP1 Antibody is a Rabbit Polyclonal antibody against NPHP1. 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.
Research Articles on Nephrocystin 1 (NPHP1)
| Target | Nephrocystin 1 (NPHP1) |
| Clonality | Polyclonal |
| Reactivity | Human, Mouse, Rat |
| Tested Applications | WB, IHC, IF/ICC |
| Host | Rabbit |
| Recommended dilutions | WB: 1/500 - 1/1000, IHC-P: 1/50 - 1/200, IF/ICC: 1/50 - 1/200. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Conjugation | Unconjugated |
| Immunogen | Recombinant fusion protein containing a sequence corresponding to amino acids 463-732 of human NPHP1. |
| Isotype | IgG |
| Form | Liquid |
| Purification | Purified by affinity chromatography. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| UniProt Primary AC | O15259 (UniProt, ExPASy) |
| UniProt Secondary AC | O14837 |
| UniProt Entry Name | NPHP1_HUMAN |
| Gene Symbol | NPHP1 |
| GeneID | 4867 |
| NCBI Accession | NP_001121651.1 |
| KEGG | hsa:4867 |
| String | 9606.ENSP00000313169 |
| Molecular Weight | Calculated MW: 83 kDa Observed MW: 83 kDa |
| Buffer | PBS, pH 7.3, containing 0.05% Proclin-300, 50% glycerol. |
| Concentration | 0.95 mg/ml |
| Availability | Shipped within 5-10 working days. |
| Note | This product is for research use only. |