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anti-NPHS1 antibody :: Rabbit NPHS1 Polyclonal Antibody

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Catalog # MBS2526145
Unit / Price
  0.02 mL  /  $110 +1 FREE 8GB USB
  0.06 mL  /  $150 +1 FREE 8GB USB
  0.12 mL  /  $225 +1 FREE 8GB USB
  0.2 mL  /  $360 +1 FREE 8GB USB
Product Name

NPHS1, Polyclonal Antibody

Popular Item
Full Product Name

NPHS1 Polyclonal Antibody

Product Synonym Names
CNF, NPHN, nephrin
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
OMIM
256300
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen affinity purification
Concentration
1.4mg/mL (lot specific)
Immunogen
Synthetic peptide of human NPHS1
Calculated Molecular Weight: 135kDa
Buffer
PBS with 0.05% sodium azide, 50% glycerol, pH7.3
Preparation and Storage
Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.
ISO Certification
Manufactured in an ISO 9001:2008 Certified Laboratory.
Other Notes
Small volumes of anti-NPHS1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-NPHS1 antibody
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.
Applications Tested/Suitable for anti-NPHS1 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-NPHS1 antibody
WB: 1:500-1:2000
IHC: 1:25-1:100

Western Blot (WB) of anti-NPHS1 antibody
Western Blot analysis of Mouse kidney tissue using NPHS1 Polyclonal Antibody at dilution of 1:700
anti-NPHS1 antibody Western Blot (WB) (WB) image
Immunohistochemistry (IHC) of anti-NPHS1 antibody
Immunohistochemistry of paraffin-embedded Human brain using NPHS1 Polyclonal Antibody at dilution of 1:40
anti-NPHS1 antibody Immunohistochemistry (IHC) (IHC) image
Immunohistochemistry (IHC) of anti-NPHS1 antibody
Immunohistochemistry of paraffin-embedded Human liver cancer using NPHS1 Polyclonal Antibody at dilution of 1:40
anti-NPHS1 antibody Immunohistochemistry (IHC) (IHC) image
NCBI/Uniprot data below describe general gene information for NPHS1. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
130,546 Da
NCBI Official Full Name
nephrin
NCBI Official Synonym Full Names
nephrosis 1, congenital, Finnish type (nephrin)
NCBI Official Symbol
NPHS1  [Similar Products]
NCBI Official Synonym Symbols
CNF; NPHN; nephrin
  [Similar Products]
NCBI Protein Information
nephrin; renal glomerulus-specific cell adhesion receptor
UniProt Protein Name
Nephrin
UniProt Synonym Protein Names
Renal glomerulus-specific cell adhesion receptor
Protein Family
UniProt Gene Name
NPHS1  [Similar Products]
UniProt Synonym Gene Names
UniProt Entry Name
NPHN_HUMAN
NCBI Summary for NPHS1
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 Comments for NPHS1
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: Immunoglobulin superfamily; Membrane protein, integral; Cell adhesion

Chromosomal Location of Human Ortholog: 19q13.1

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

Molecular Function: protein domain specific binding; protein binding; spectrin binding; myosin 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
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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