ITT3037
ITT3037
ITT3037
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- Catalog: ITT3037
- Gene/Protein: NPHP4
- Product Description: Immunotag™ Nephrocystin-4 Polyclonal Antibody
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Description
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Specifications
Antibody Specification Datasheet 
IMPORTANT NOTE This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. Target Protein NEPHrocystin-4 Clonality Polyclonal Storage/Stability -20°C/1 year Application IHC-p,ELISA Recommended Dilution Immunohistochemistry: 1/100 - 1/300. ELISA: 1/40000. Not yet tested in other applications. Concentration 1 mg/ml Reactive Species Human Host Species Rabbit Immunogen The antiserum was produced against synthesized peptide derived from human NPHP4. AA range:877-926 Specificity Nephrocystin-4 Polyclonal Antibody detects endogenous levels of Nephrocystin-4 protein. Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Gene Name NPHP4 Accession No. O75161 P59240 Alternate Names NPHP4; KIAA0673; Nephrocystin-4; Nephroretinin Description nephrocystin 4(NPHP4) Homo sapiens This gene encodes a protein involved in renal tubular development and function. This protein interacts with nephrocystin, and belongs to a multifunctional complex that is localized to actin- and microtubule-based structures. Mutations in this gene are associated with nephronophthisis type 4, a renal disease, and with Senior-Loken syndrome type 4, a combination of nephronophthisis and retinitis pigmentosa. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2014], Protein Expression Brain,Hippocampus,Kidney,Testis, Subcellular Localization nucleus,centrosome,cytosol,cytoskeleton,cell-cell junction,bicellular tight junction,membrane,nonmotile primary cilium,photoreceptor connecting cilium,ciliary transition zone,ribbon synapse,ciliary base, Protein Function disease:Defects in NPHP4 are the cause of nephronophthisis type 4 (NPHP4) [MIM:606966]; also known as familial juvenile nephronophthisis 4. NPHP4 is an autosomal recessive inherited disease resulting in end-stage renal disease at age ranging between 6 and 35 years. It is a progressive tubulo-interstitial kidney disorder characterized by polydipsia, polyuria, anemia and growth retardation. The most prominent histological features are modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts.,disease:Defects in NPHP4 are the cause of Senior-Loken syndrome type 4 (SLSN4) [MIM:606996]. SLSN is a renal-retinal disorder characterized by progressive wasting of the filtering unit of the kidney, with or without medullary cystic renal disease, and progressive eye disease. Typically this disorder becomes apparent during the first year of life.,similarity:Belongs to the NPHP4 family.,subunit:Interacts with NPHP1 and RPGRIP1L.,tissue specificity:Expressed in kidney, skeletal muscle, heart and liver, and to a lesser extent in brain and lung., Usage For Research Use Only! Not for diagnostic or therapeutic procedures. - Reviews
| Antibody Specification | |
| Datasheet | |
| IMPORTANT NOTE | This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. |
| Target Protein | NEPHrocystin-4 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | IHC-p,ELISA |
| Recommended Dilution | Immunohistochemistry: 1/100 - 1/300. ELISA: 1/40000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human |
| Host Species | Rabbit |
| Immunogen | The antiserum was produced against synthesized peptide derived from human NPHP4. AA range:877-926 |
| Specificity | Nephrocystin-4 Polyclonal Antibody detects endogenous levels of Nephrocystin-4 protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | NPHP4 |
| Accession No. | O75161 P59240 |
| Alternate Names | NPHP4; KIAA0673; Nephrocystin-4; Nephroretinin |
| Description | nephrocystin 4(NPHP4) Homo sapiens This gene encodes a protein involved in renal tubular development and function. This protein interacts with nephrocystin, and belongs to a multifunctional complex that is localized to actin- and microtubule-based structures. Mutations in this gene are associated with nephronophthisis type 4, a renal disease, and with Senior-Loken syndrome type 4, a combination of nephronophthisis and retinitis pigmentosa. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2014], |
| Protein Expression | Brain,Hippocampus,Kidney,Testis, |
| Subcellular Localization | nucleus,centrosome,cytosol,cytoskeleton,cell-cell junction,bicellular tight junction,membrane,nonmotile primary cilium,photoreceptor connecting cilium,ciliary transition zone,ribbon synapse,ciliary base, |
| Protein Function | disease:Defects in NPHP4 are the cause of nephronophthisis type 4 (NPHP4) [MIM:606966]; also known as familial juvenile nephronophthisis 4. NPHP4 is an autosomal recessive inherited disease resulting in end-stage renal disease at age ranging between 6 and 35 years. It is a progressive tubulo-interstitial kidney disorder characterized by polydipsia, polyuria, anemia and growth retardation. The most prominent histological features are modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts.,disease:Defects in NPHP4 are the cause of Senior-Loken syndrome type 4 (SLSN4) [MIM:606996]. SLSN is a renal-retinal disorder characterized by progressive wasting of the filtering unit of the kidney, with or without medullary cystic renal disease, and progressive eye disease. Typically this disorder becomes apparent during the first year of life.,similarity:Belongs to the NPHP4 family.,subunit:Interacts with NPHP1 and RPGRIP1L.,tissue specificity:Expressed in kidney, skeletal muscle, heart and liver, and to a lesser extent in brain and lung., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |