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Western Blot (WB) (Western blot analysis of Nephrin in human kidney tissue lysate with Nephrin antibody at 1 μg/ml in (A) the absence and (B) the presence of blocking peptide.)

Rabbit anti-Human Nephrin Polyclonal Antibody | anti-NPHS1 antibody

Nephrin Antibody

Gene Names
NPHS1; CNF; NPHN; nephrin
Reactivity
Human
Applications
ELISA, Western Blot
Purity
Nephrin antibody is affinity chromatography purified via peptide column.
Synonyms
Nephrin; Polyclonal Antibody; Nephrin Antibody; NPHN; NPHS1; Renal glomerulus-specific cell adhesion receptor3; nephrosis 1; congenital; Finnish type (nephrin); anti-NPHS1 antibody
Ordering
For Research Use Only!
Host
Rabbit
Reactivity
Human
Clonality
Polyclonal
Isotype
IgG
Specificity
Nephrin antibody is human specific.
Purity/Purification
Nephrin antibody is affinity chromatography purified via peptide column.
Form/Format
Liquid
Concentration
1 mg/mL (varies by lot)
Sequence Length
1241
Applicable Applications for anti-NPHS1 antibody
ELISA (EIA), Western Blot (WB)
Application Notes
Nephrin antibody can be used for detection of Nephrin by Western blot at 1 - 2 mug/ml.
Conjugate
Unconjugated
Immunogen
Nephrin antibody was raised against a 15 amino acid peptide near the carboxy terminus of human Nephrin.
Buffer
Nephrin antibody is supplied in PBS containing 0.02% sodium azide.
Preparation and Storage
Nephrin antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year.

Western Blot (WB)

(Western blot analysis of Nephrin in human kidney tissue lysate with Nephrin antibody at 1 μg/ml in (A) the absence and (B) the presence of blocking peptide.)

Western Blot (WB) (Western blot analysis of Nephrin in human kidney tissue lysate with Nephrin antibody at 1 μg/ml in (A) the absence and (B) the presence of blocking peptide.)

Immunohistochemistry (IHC)

(Immunohistochemistry of Nephrin in human kidney tissue with Nephrin antibody at 5 μg/ml.)

Immunohistochemistry (IHC) (Immunohistochemistry of Nephrin in human kidney tissue with Nephrin antibody at 5 μg/ml.)

Immunofluorescence (IF)

(Immunofluorescence of Nephrin in human kidney tissue with Nephrin antibody at 20 μg/ml.)

Immunofluorescence (IF) (Immunofluorescence of Nephrin in human kidney tissue with Nephrin antibody at 20 μg/ml.)
Related Product Information for anti-NPHS1 antibody
Nephrin is strongly expressed in renal glomeruli and is a member of the immunoglobulin family of cell adhesion molecules. Mutations in the Nephrin gene result in congenital nephrotic syndrome, an autosomal-recessive disorder characterized by massive proteinuria in utero and nephrosis at birth (1). Renal glomeruli allow normal kidneys to filter plasma so that it is very pure. Nephrin is expressed in the podocyte slit-diaphragm of the renal glomeruli in a manner that suggests that Nephrin molecules homodimerize in an anti-parallel fashion similar to cadherin interactions in adherens junctions. Thus, Nephrin may constitute the entire extracellular structure of the slit-diaphragm (2,3).

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
Molecular Weight
Predicted: 125; 137 kDa

Observed: 125 kDa
NCBI Official Full Name
nephrin
NCBI Official Synonym Full Names
nephrosis 1, congenital, Finnish type (nephrin)
NCBI Official Symbol
NPHS1
NCBI Official Synonym Symbols
CNF; NPHN; nephrin
NCBI Protein Information
nephrin; renal glomerulus-specific cell adhesion receptor
UniProt Protein Name
Nephrin
Protein Family
UniProt Gene Name
NPHS1
UniProt Synonym Gene Names
NPHN
UniProt Entry Name
NPHN_HUMAN

NCBI Description

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.[provided by RefSeq, Oct 2009]

Uniprot Description

NPHS1: Seems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion. Interacts with CD2AP (via C-terminal domain). Interacts with MAGI1 (via PDZ 2 and 3 domains) forming a tripartite complex with IGSF5/JAM4. Interacts with DDN; the interaction is direct. Self-associates (via the Ig-like domains). Also interacts (via the Ig-like domains) with KIRREL/NEPH1 and KIRREL2; the interaction with KIRREL is dependent on KIRREL glycosylation. Forms a complex with ACTN4, CASK, IQGAP1, MAGI2, SPTAN1 and SPTBN1. Interacts with NPHS2. Specifically expressed in podocytes of kidney glomeruli. Belongs to the immunoglobulin superfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cell adhesion; Immunoglobulin superfamily; Membrane protein, integral

Chromosomal Location of Human Ortholog: 19q13.1

Cellular Component: cell projection; protein complex; integral to plasma membrane; plasma membrane; lipid raft

Molecular Function: protein domain specific binding; protein binding; myosin binding; spectrin binding; alpha-actinin binding

Biological Process: skeletal muscle development; positive regulation of actin filament polymerization; glomerular basement membrane development; JNK cascade; excretion; cell adhesion; myoblast fusion

Disease: Nephrotic Syndrome, Type 1

Research Articles on NPHS1

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Product Notes

The NPHS1 nphs1 (Catalog #AAA153616) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Nephrin Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Nephrin can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB). Nephrin antibody can be used for detection of Nephrin by Western blot at 1 - 2 mug/ml. Researchers should empirically determine the suitability of the NPHS1 nphs1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Nephrin, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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