Rabbit anti-Human, Mouse NPHS1 Polyclonal Antibody | anti-NPHS1 antibody

NPHS1 (Nephrosis 1 Congenital Finnish Type, CNF, NPHN, Nephrin) (HRP)

Cat. Num. AAA6429492

• Gene Names: NPHS1; CNF; NPHN; nephrin
• Reactivity: Human, Mouse
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified by affinity chromatography.
• Synonyms: NPHS1; Polyclonal Antibody; NPHS1 (Nephrosis 1 Congenital Finnish Type; CNF; NPHN; Nephrin) (HRP); anti-NPHS1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes NPHS1. Species Crossreactivity: human, mouse
• Purity/Purification: Purified by affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish Peroxidase (HRP).
• Concentration: ~1mg/ml (varies by lot)

• Applicable Applications for anti-NPHS1 antibody: Immunohistochemistry (IHC), Western Blot (WB)
• Application Notes: IHC: 1:50-1:100; WB: 1:500-1:2000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide corresponding to human NPHS1.
• Conjugate: HRP
• Note: Preservative Free
• 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. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using NPHS1 antibody. (1: Mouse spleen tissue lysate 2: Mouse kidney tissue lysate))

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded Human liver cancer tissue using NPHS1 antibody.)

Product Categories/Family for anti-NPHS1 antibody

Antibodies; Nephrin

NCBI and Uniprot Product Information

• NCBI GI #: 10441644
• NCBI GeneID: 4868
• UniProt Accession #: O60500; A6NDH2; C3RX61
• Molecular Weight: 134,742 Da
• NCBI Official Full Name: nephrin
• NCBI Official Synonym Full Names: nephrosis 1, congenital, Finnish type (nephrin)
• NCBI Official Symbol: NPHS1
• NCBI Official Synonym Symbols: CNF; NPHN; nephrin
• NCBI Protein Information: nephrin; renal glomerulus-specific cell adhesion receptor
• UniProt Protein Name: Nephrin
• Protein Family: Nephrin
• 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