Nephrin Rabbit Polyclonal Antibody
Catalog# ER1803-67
Nephrin Rabbit Polyclonal Antibody
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WB
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IF-Cell
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Human
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Rat
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unconjugated
Overview
Product Name
Nephrin Rabbit Polyclonal Antibody
Antibody Type
Rabbit Polyclonal Antibody
Immunogen
Recombinant protein within Human Nephrin aa 1,042-1,241 / 1,241.
Species Reactivity
Human, Rat
Validated Applications
WB, IF-Cell
Target Molecular Weight
Predicted band size: 135 kDa
Positive Control
Human kidney tissue lysates, 293T, PANC-1, PC-12.
Conjugation
unconjugated
RRID
Product Features
Form
Liquid
Concentration
1 mg/ml.(The concentration of this product may be batch-dependent)
Storage Instructions
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). It is recommended to aliquot into single-use upon delivery. Store at -20℃ long term.
Storage Buffer
1*PBS (pH7.4), 0.2% BSA, 50% Glycerol. Preservative: 0.05% Sodium Azide.
Isotype
IgG
Purification Method
Immunogen affinity purified.
Application Dilution
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WB
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1:1,000-1:2,000
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IF-Cell
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1:50-1:200
Target
Function
This gene encodes a member of the immunoglobulin family of cell adhesion molecules that functions in the glomerular filtration barrier in the kidney. The renal filtration barrier consists of fenestrated endothelial cells, the glomerular basement membrane, and the podocytes of epithelial cells. 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. A defect in the gene for nephrin, NPHS1, is associated with congenital nephrotic syndrome of the Finnish type and causes massive amounts of protein to be leaked into the urine, or proteinuria. Nephrin is also required for cardiovascular development. Plays a role in skeletal muscle formation through regulation of myoblast fusion.
Background References
1. Holthoefer H et al. Nephrin localizes at the podocyte filtration slit area and is characteristically spliced in the human kidney. Am J Pathol 155:1681-1687 (1999).
2. Ruotsalainen V et al. Nephrin is specifically located at the slit diaphragm of glomerular podocytes. Proc Natl Acad Sci USA 96:7962-7967 (1999).
Sequence Similarity
Belongs to the immunoglobulin superfamily.
Tissue Specificity
Specifically expressed in podocytes of kidney glomeruli.
Post-translational Modification
Phosphorylated at Tyr-1193 by FYN, leading to the recruitment and activation of phospholipase C-gamma-1/PLCG1.
Subcellular Location
Cell membrane.
Synonyms
CNF antibody
Nephrin antibody
Nephrosis 1 congenital Finnish type antibody
Nephrosis 1, congenital, Finnish type (nephrin) antibody
NPHN antibody
NPHN_HUMAN antibody
NPHS 1 antibody
Nphs1 antibody
Renal glomerulus specific cell adhesion receptor antibody
Renal glomerulus-specific cell adhesion receptor antibody
Images
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Western blot analysis of Nephrin on human kidney tissue lysates with Rabbit anti-Nephrin antibody (ER1803-67) at 1/1,000 dilution.
Lysates/proteins at 30 µg/Lane.
Predicted band size: 135 kDa
Observed band size: 200 kDa
Exposure time: 30 seconds; ECL: K1801;
4-20% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (ER1803-67) at 1/1,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature. -
ICC staining Nephrin in 293T cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 1% Blocker BSA for 15 minutes at room temperature. Cells were probed with Nephrin polyclonal antibody at a dilution of 1:100 for at least 1 hour at room temperature, washed with PBS. Alexa Fluorc™ 488 Goat anti-Rabbit IgG was used as the secondary antibody at 1/100 dilution. The nuclear counter stain is DAPI (blue).
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ICC staining Nephrin in PANC-1 cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 1% Blocker BSA for 15 minutes at room temperature. Cells were probed with Nephrin polyclonal antibody at a dilution of 1:100 for at least 1 hour at room temperature, washed with PBS. Alexa Fluorc™ 488 Goat anti-Rabbit IgG was used as the secondary antibody at 1/100 dilution. The nuclear counter stain is DAPI (blue).
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ICC staining Nephrin in PC-12 cells (green). Formalin fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 10 minutes at room temperature and blocked with 1% Blocker BSA for 15 minutes at room temperature. Cells were probed with Nephrin polyclonal antibody at a dilution of 1:100 for at least 1 hour at room temperature, washed with PBS. Alexa Fluorc™ 488 Goat anti-Rabbit IgG was used as the secondary antibody at 1/100 dilution. The nuclear counter stain is DAPI (blue).
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"
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