Nephrocystin-4 Rabbit Polyclonal Antibody
Konjugasyon: Unconjugated
Rabbit polyclonal Antibody
Uygulama
Reaktivite
Human,Rat,Mouse
Gen Adı
NPHP4
Saklama
Aliquot and store at -20°C (valid for 12 months). Avoid freeze/thaw cycles.
Özet
| Ürün Adı | Nephrocystin-4 Rabbit Polyclonal Antibody |
| Açıklama | Rabbit polyclonal Antibody |
| Konak | Rabbit |
| Reaktivite | Human,Rat,Mouse |
| Konjugasyon | Unconjugated |
| Modifikasyon | Unmodified |
| İzotip | IgG |
| Klonalite | Polyclonal |
| Form | Liquid |
| Konsantrasyon | Unconjugated |
| Saklama | Aliquot and store at -20°C (valid for 12 months). Avoid freeze/thaw cycles. |
| Nakliye | Ice bags. |
| Tampon | Liquid in PBS containing 50% glycerol, 0.5% protective protein and 0.02% New type preservative N. |
| Saflaştırma | Affinity purification |
Antijen Bilgisi
| Gen Adı | NPHP4 |
| Alternatif İsimler | NPHP4; KIAA0673; Nephrocystin-4; Nephroretinin |
| Gen Kimliği | 261734 |
| SwissProt Kimliği | O75161 |
| İmmünojen | The antiserum was produced against synthesized peptide derived from human NPHP4. AA range:877-926 |
Uygulama
| Uygulama | IHC,ICC/IF,ELISA |
| Seyreltme Oranı | IHC 1:100-1:300,ICC/IF 1:50-1:200,ELISA 1:20000-1:40000 |
| Moleküler Ağırlık | - |
Araştırma Alanı
Arka Plan
| 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],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., |
