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Testing Data (Detection limit for recombinant GST tagged NPHS1 is 0.1 ng/ml as a capture antibody.)

Mouse NPHS1 Monoclonal Antibody | anti-NPHS1 antibody

NPHS1 (Nephrosis 1, Congenital, Finnish Type (nephrin), CNF, NPHN) (Biotin)

Gene Names
NPHS1; CNF; NPHN; nephrin
Applications
Western Blot
Purity
Purified
Synonyms
NPHS1; Monoclonal Antibody; NPHS1 (Nephrosis 1; Congenital; Finnish Type (nephrin); CNF; NPHN) (Biotin); Nephrosis 1; NPHN; anti-NPHS1 antibody
Ordering
For Research Use Only!
Host
Mouse
Clonality
Monoclonal
Isotype
IgG2a,k
Clone Number
3D2
Specificity
Recognizes NPHS1.
Purity/Purification
Purified
Form/Format
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
Applicable Applications for anti-NPHS1 antibody
Western Blot (WB)
Application Notes
Applications are based on unconjugated antibody.
Immunogen
NPHS1 (NP_004637.1, 33aa-122aa) partial recombinant protein with GST tag. MW of the GST tag alone is 26kD.
Immunogen Sequence
GFWALPENLTVVEGASVELRCGVSTPGSAVQWAKDGLLLGPDPRIPGFPRYRLEGDPARGEFHLHIEACDLSDDAEYECQVGRSEMGPEL
Conjugate
Biotin
Preparation and Storage
Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Testing Data

(Detection limit for recombinant GST tagged NPHS1 is 0.1 ng/ml as a capture antibody.)

Testing Data (Detection limit for recombinant GST tagged NPHS1 is 0.1 ng/ml as a capture antibody.)
Related Product Information for anti-NPHS1 antibody
Nephrin is a kidney glomerular filtration barrier protein that is an essential component of the interpodocyte-spanning slit diaphragm. Mutations in the nephrin gene are associated with congenital nephrotic syndrome (NPHS1; MIM 256300). [supplied by OMIM]
Product Categories/Family for anti-NPHS1 antibody

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
Molecular Weight
130,546 Da
NCBI Official Full Name
nephrin
NCBI Official Synonym Full Names
NPHS1, nephrin
NCBI Official Symbol
NPHS1
NCBI Official Synonym Symbols
CNF; NPHN; nephrin
NCBI Protein Information
nephrin
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: Immunoglobulin superfamily; Cell adhesion; 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 #AAA6174364) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's NPHS1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Applications are based on unconjugated antibody. 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 "NPHS1, Monoclonal 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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