Rabbit Anti-NPHP1 Polyclonal Antibody
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产品编号
PAV2572
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别名
nephrocystin 1, JBTS4, NPH1, SLSN1
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目录价格
InquiryRMB
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规格
- 100ul
产品介绍
| Alias | nephrocystin 1, JBTS4, NPH1, SLSN1 |
| Catalog Number | PAV2572 |
| Product Description | Rabbit polyclonal antibody raised against human NPHP1 (Nephrocystin-1). Affinity purified from rabbit antiserum by antigen-specific affinity chromatography. Validated applications: WB, ELISA. Supplied as liquid in PBS containing 50% glycerol and 0.02% sodium azide at 1 mg/ml. |
| Molecular Name | NPHP1 |
| Species | Human, Mouse |
| Host | Rabbit |
| Size | 100ul |
| Clonity | Polyclonal |
| Protein Family | 细胞黏附 |
| Concentration | 1 mg/ml |
| Purification | affinity-chromatography |
| Formulation | supply in PBS containing 50% glycerol, and 0.02% sodium azide. |
| 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. [provided by RefSeq, Jul 2008] |
| Immunogen | This antibody was raised against from part region of human NPHP1 protein (UniProt: O15259) |
| Application | WB, ELISA |
| Recommended Dilution | WB 1:500-2000 ELISA 1:5000-20000 |
| Gene ID | 4867 |
| Tag | Unconjugated |
| Cross-reactivity | Human, Mouse |
| Predicted Molecular Weight | ≈83.3 kDa |
| Storage Condition | Store at 4℃ for for short term storage. It is recommended to aliquote to ~10ul per vial and store at -20℃ for long term storage. Avoid repeated freezing and thawing cycles. |
| Shipping Condition | This product is shipped on blue ice. |
| Molecular Weight | 83.3 kDa |
| Cross-reactivity | Human, Mouse |
| Uniprot ID | O15259 |
| Note | Gene ID: 4867; UniProt: O15259; Chromosome: 2q13; Predicted MW: 83.3 kDa |
| Function | Together with BCAR1 it may play a role in the control of epithelial cell polarity (By similarity). Involved in the organization of apical junctions in kidney cells together with NPHP4 and RPGRIP1L/NPHP8 (By similarity). Does not seem to be strictly required for ciliogenesis (By similarity). Seems to help to recruit PTK2B/PYK2 to cell matrix adhesions, thereby initiating phosphorylation of PTK2B/PYK2 and PTK2B/PYK2-dependent signaling (By similarity). May play a role in the regulation of intraflagellar transport (IFT) during cilia assembly. Required for normal retina development (By similarity). In connecting photoreceptor cilia influences the movement of some IFT proteins such as IFT88 and WDR19. Involved in spermatogenesis (By similarity). |
| Product Declaration | 本产品仅用于研究用途(RUO)。不能用于诊断或直接用于人体实验。 |
| Product Full Name | Rabbit Anti-Human NPHP1 Polyclonal Antibody |
| Target | NPHP1 |
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