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anti-NPHS1 antibody :: Rabbit NPHS1 Polyclonal Antibody

Scan QR to view Datasheet 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
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

NPHS1, Polyclonal Antibody

★Popular Item★
 Also Known As   

NPHS1 Polyclonal Antibody

 Product Synonym Names    CNF, NPHN, nephrin
 Product Gene Name   

anti-NPHS1 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    256300
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Purity/Purification    Antigen affinity purification
 Concentration    1.4mg/mL (lot specific)
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 Immunogen    Synthetic peptide of human NPHS1
 Calculated Molecular Weight: 135kDa   
 Buffer    PBS with 0.05% sodium azide, 50% glycerol, pH7.3
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 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.
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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
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 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
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 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
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 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
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NCBI/Uniprot data below describe general gene information for NPHS1. It may not necessarily be applicable to this product.
 NCBI GI #    4758822
 NCBI GeneID    4868
 NCBI Accession #    NP_004637.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_004646.3 [Other Products]
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 UniProt Secondary Accession #    A6NDH2; C3RX61 [Other Products]
 UniProt Related Accession #    O60500 [Other Products]
 Molecular Weight    130,546 Da
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 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    Nephrin
 UniProt Gene Name    NPHS1 [Similar Products]
 UniProt Synonym Gene Names    NPHN [Similar Products]
 UniProt Entry Name    NPHN_HUMAN
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 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]
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 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
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 Research Articles on NPHS1    1. No pathogenic NPHS1 mutations were found in the cohort of children with steroid-resistant focal segmental glomerulosclerosis.
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 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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Products associated with anti-NPHS1 antibodyPathways associated with anti-NPHS1 antibody
 Reference Product  PubMed Publications
 NPHS2 antibody  >78 publications with NPHS1 and NPHS2
 CD2AP antibody  >24 publications with NPHS1 and CD2AP
 TRPC6 antibody  >8 publications with NPHS1 and TRPC6
 TJP1 antibody  >6 publications with NPHS1 and TJP1
 SRC antibody  >4 publications with NPHS1 and SRC
 KIRREL antibody  >4 publications with NPHS1 and KIRREL
 FYN antibody  >1 publications with NPHS1 and FYN
 Products by Pathway  Pathway Diagram
 Cell-Cell Communication Pathway antibodies  Cell-Cell Communication Pathway Diagram
 Nephrin Interactions Pathway antibodies  Nephrin Interactions Pathway Diagram
 Nephrin/Neph1 Signaling In The Kidney Podocyte Pathway antibodies  Nephrin/Neph1 Signaling In The Kidney Podocyte Pathway Diagram
Diseases associated with anti-NPHS1 antibodyOrgans/Tissues associated with anti-NPHS1 antibody
 Disease Name  Pubmed Publications
 Nephrotic Syndrome Antibodies  >143 publications with NPHS1 and Nephrotic Syndrome
 Kidney Diseases Antibodies  >142 publications with NPHS1 and Kidney Diseases
 Nephrosis Antibodies  >112 publications with NPHS1 and Nephrosis
 Proteinuria Antibodies  >76 publications with NPHS1 and Proteinuria
 Glomerulonephritis Antibodies  >37 publications with NPHS1 and Glomerulonephritis
 Glomerulosclerosis, Focal Segmental Antibodies  >37 publications with NPHS1 and Glomerulosclerosis, Focal Segmental
 Renal Insufficiency Antibodies  >16 publications with NPHS1 and Renal Insufficiency
 Kidney Failure, Chronic Antibodies  >15 publications with NPHS1 and Kidney Failure, Chronic
 Disease Models, Animal Antibodies  >12 publications with NPHS1 and Disease Models, Animal
 Hypertension Antibodies  >9 publications with NPHS1 and Hypertension
 Organ/Tissue Name  Pubmed Publications
 Kidney Antibodies  >131 publications with NPHS1 and Kidney
 Pancreas Antibodies  >8 publications with NPHS1 and Pancreas
 Brain Antibodies  >6 publications with NPHS1 and Brain
 Thymus Antibodies  >3 publications with NPHS1 and Thymus
 Testis Antibodies  >2 publications with NPHS1 and Testis
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