Human nephrosis 1, congenital, Finnish type (nephrin) ELISA Kit | NPHS1 elisa kit

Human Nephrin ELISA Kit

Cat. Num. AAA700868

• Gene Names: NPHS1; CNF; NPHN; nephrin
• Reactivity: Human
• Synonyms: nephrosis 1; congenital; Finnish type (nephrin); Human Nephrin ELISA Kit; CNF; NPHN; nephrin; renal glomerulus-specific cell adhesion receptor; NPHS1 elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of Human NPHS1. No significant cross-reactivity or interference between Human NPHS1 and analogues was observed.
• Sequence Length: 1241

• Samples: Serum, plasma, tissue homogenates
• Detection Range: 0.156 ng/ml-10 ng/ml
• Sensitivity: 0.039 ng/ml
• Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV% is less than 8%; Three samples of known concentration were tested twenty times on one plate to assess.; Inter-assay Precision (Precision between assays): CV% is less than 10%; Three samples of known concentration were tested in twenty assays to assess.
• Detection Wavelength: 450 nm
• Sample Volume: 50-100ul
• Protein Biological Process 1: Cell Adhesion
• Protein Biological Process 3: Cell adhesion
• Preparation and Storage: Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for NPHS1 elisa kit

Introduction: Nephrin is a protein necessary for the proper functioning of therenal filtration barrier. The renal filtration barrier consists offenestrated endothelial cells, the glomerular basementmembrane, and the podocytes of epithelial cells. Nephrin is atransmembrane protein involved with the slit diaphragm. A defectin the gene for nephrin is associated with congenital nephroticsyndrome of the Finnish type and causes massive amounts ofprotein to be leaked into the urine, or proteinuria.

Principle of the Assay: The microtiter plate provided in this kit has been pre-coated withan antibody specific to nephrin. Standards or samples are thenadded to the appropriate microtiter plate wells with abiotin-conjugated antibody preparation specific for nephrin andAvidin conjugated to Horseradish Peroxidase (HRP) is added toeach microplate well and incubated. Then a TMB (3,3',5,5' tetramethyl-benzidine) substrate solution is added to each well.Only those wells that contain nephrin, biotin-conjugated antibodyand enzyme-conjugated Avidin will exhibit a change in color. Theenzyme-substrate reaction is terminated by the addition of asulphuric acid solution and the color change is measuredspectrophotometrically at a wavelength of 450 nm +/- 2 nm. Theconcentration of nephrin in the samples is then determined bycomparing the O.D. of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 4758822
• NCBI GeneID: 4868
• NCBI Accession #: NP_004637.1
• NCBI GenBank Nucleotide #: NM_004646.3
• 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

Function: 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