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Nephronophthisis Antibody

Novus Biologicals, part of Bio-Techne | Catalog # NBP1-59110

Novus Biologicals, part of Bio-Techne
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NBP1-59110

Key Product Details

Species Reactivity

Human

Applications

Western Blot

Label

Unconjugated

Antibody Source

Polyclonal Rabbit IgG

Concentration

0.5 mg/ml

Product Specifications

Immunogen

Synthetic peptides corresponding to NPHP1(nephronophthisis 1 (juvenile)) The peptide sequence was selected from the middle region of NPHP1. Peptide sequence GILFELGISYIRNSTGERGELSCGWVFLKLFDASGVPIPAKTYELFLNGG. The peptide sequence for this immunogen was taken from within the described region.

Clonality

Polyclonal

Host

Rabbit

Isotype

IgG

Description

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.

Scientific Data Images for Nephronophthisis Antibody

Western Blot: Nephronophthisis Antibody [NBP1-59110]

Western Blot: Nephronophthisis Antibody [NBP1-59110]

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

Applications for Nephronophthisis Antibody

Application
Recommended Usage

Western Blot

1.0 ug/ml

Formulation, Preparation, and Storage

Purification

Affinity purified

Formulation

PBS, 2% Sucrose

Preservative

0.09% Sodium Azide

Concentration

0.5 mg/ml

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.

Background: Nephronophthisis

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.

Alternate Names

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

Gene Symbol

NPHP1

Additional Nephronophthisis Products

Product Documents for Nephronophthisis Antibody

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Product Specific Notices for Nephronophthisis Antibody

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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