Nephronophthisis Antibody

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Western Blot: Nephronophthisis Antibody [NBP1-59110] - Human Heart lysate, concentration 0.2-1 ug/ml.

Product Details

Summary
Reactivity Hu, Mu, Rt, Po, Bv, Ca, Eq, GP, Rb, ZeSpecies Glossary
Applications WB
Clonality
Polyclonal
Host
Rabbit
Conjugate
Unconjugated

Order Details

Nephronophthisis Antibody Summary

Immunogen
Synthetic peptides corresponding to NPHP1(nephronophthisis 1 (juvenile)) The peptide sequence was selected from the middle region of NPHP1. Peptide sequence GILFELGISYIRNSTGERGELSCGWVFLKLFDASGVPIPAKTYELFLNGG.
Isotype
IgG
Clonality
Polyclonal
Host
Rabbit
Gene
NPHP1
Purity
Immunogen affinity purified
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Applications/Dilutions

Dilutions
  • Western Blot 1:100-1:2000
Application Notes
This is a rabbit polyclonal antibody against NPHP1 and was validated on Western blot.

Packaging, Storage & Formulations

Storage
Store at -20C. Avoid freeze-thaw cycles.
Buffer
PBS and 2% Sucrose
Preservative
0.09% Sodium Azide
Purity
Immunogen affinity purified

Notes

The addition of 50% glycerol is optional for those storing this antibody at -20C and not aliquoting smaller units. However, please note that glycerol may interrupt some downstream antibody applications and should be added with caution.

Alternate Names for Nephronophthisis Antibody

  • FLJ97602
  • JBTS4NPH1Juvenile nephronophthisis 1 protein
  • nephrocystin 1
  • nephrocystin-1
  • nephronophthisis 1 (juvenile)
  • SLSN1

Background

Together with Cas NPHP1 may play a role in the control of epithelial cell polarity. NPHP1 seems to help to recruit protein tyrosine kinase 2 beta (PTK2B) to cell matrix adhesions, thereby initiating phosphorylation of PTK2B and PTK2B-dependent signaling.This gene encodes a protein with src homology domain 3 (SH3) patterns. This protein interacts with Crk-associated substrate, and it appears to function in the control of cell division, as well as in cell-cell and cell-matrix adhesion signaling, likely as part of a multifunctional complex localized in actin- and microtubule-based structures. Mutations in this gene cause familial juvenile nephronophthisis type 1, a kidney disorder involving both tubules and glomeruli. Defects in this gene are also associated with Senior-Loken syndrome type 1, also referred to as juvenile nephronophthisis with Leber amaurosis, which is characterized by kidney and eye disease, and with Joubert syndrome type 4, which is characterized by cerebellar ataxia, oculomotor apraxia, psychomotor delay and neonatal breathing abnormalities, sometimes including retinal dystrophy and renal disease. Multiple transcript variants encoding different isoforms have been found for this gene.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Video Protocols

WB Video Protocol

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Bioinformatics

Gene Symbol NPHP1
Uniprot