NPHS1 Polyclonal Antibody
规格: | / 60μL / 120μL / 200μL |
价格: | / ¥1380 / ¥2220 / ¥3300 |
货号:E-AB-70211
宿主: Rabbit
反应性: M
应用: IHC
Overview
Synonyms | CNF,Nephrin,Nephrosis 1 congenital Finnish type,Nephrosis 1,congenital,Finnish type (nephrin),NPHN,NPHN,NPHS 1,Nphs1,Renal glomerulus specific cell adhesion receptor,Renal glomerulus-specific cell adhesion receptor,NPHS1抗体 |
Swissprot | Q9QZS7 |
Source | Rabbit |
Reactivity | Mouse |
Immunogen | KLH conjugated Synthetic peptide corresponding to Mouse Nephrin |
Application | IHC(Detection kit: E-IR-R213) |
Recommended dilution | IHC,,1:1000-1:2000 |
Concentration | 1.3 mg/mL |
Clonality | Polyclonal |
Properties
Cellular localization | Cell membrane. Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane. |
Tissue specificity | Expressed in kidney glomeruli. In the embryo, expressed in the mesonephric kidney at E11 with strong expression in cranial tubules with podocyte-like structures. Expression is observed in the podocytes of the developing kidney from E13. High expression is also detected in the developing cerebellum, hindbrain, spinal cord, retina and hypothalamus. Expressed in skeletal muscle during myoblast fusion such as in the adult following acute injury and in the embryo but not detected in uninjured adult skeletal muscle. Isoform 1 and isoform 2 are expressed in the newborn brain and developing cerebellum. Isoform 1 is the predominant isoform in adult kidney.Highest expression level in glomerular tuft. |
Isotype | IgG |
Purification | Affinity purification |
Conjugation | Unconjugated |
Storage instructions | Store at -20℃. Avoid freeze / thaw cycles. |
Storage buffer | PBS with 0.02% sodium azide,100 μg/ml BSA and 50% glycerol. |
Background | This gene encodes a member of the immunoglobulin family of cell adhesion molecules that functions in the glomerular filtration barrier in the kidney. The gene is primarily expressed in renal tissues, and the protein is a type-1 transmembrane protein found at the slit diaphragm of glomerular podocytes. The slit diaphragm is thought to function as an ultrafilter to exclude albumin and other plasma macromolecules in the formation of urine. Mutations in this gene result in Finnish-type congenital nephrosis 1, characterized by severe proteinuria and loss of the slit diaphragm and foot processes.In Western blots, nephrin antibodies generated against the two terminal extracellular Ig domains of recombinant human nephrin recognized a 180-kDa protein in lysates of human glomeruli and a 150-kDa protein in transfected COS-7 cell lysates. |