Human Niemann Pick Disease Type C2 (NPC2) ELISA Kit | NPC2 elisa kit

Human Niemann Pick Disease Type C2 (NPC2) ELISA Kit

Cat. Num. AAA456186

• Gene Names: NPC2; HE1; EDDM1
• Reactivity: Human
• Synonyms: Niemann Pick Disease Type C2 (NPC2); Human Niemann Pick Disease Type C2 (NPC2) ELISA Kit; NPC2 elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of NPC2. No significant cross-reactivity or interference between NPC2 and analogues was observed.
• Sequence Length: 125

• Samples: Serum, plasma, tissue homogenates and other biological fluids.
• Assay Type: Sandwich
• Detection Range: 0.781-50ng/mL
• Sensitivity: 0.31ng/mL
• Intra-assay Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level NPC2 were tested 20 times on one plate, respectively. Intra-Assay: CV<10%
• Inter-assay Precision: Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level NPC2 were tested on 3 different plates, 8 replicates in each plate. CV(%) = SD/meanX100. Inter-Assay: CV<12%
• Preparation and Storage: For unopened kit, all reagents should be kept according to the labels on vials. The TMB Substrate, Wash Buffer, Stop Solution should be stored at 4 degree C. All others should be stored at -20 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for NPC2 elisa kit

Intended Uses: The kit is a sandwich enzyme immunoassay for in vitro quantitative measurement of NPC2 in human serum, plasma, tissue homogenates and other biological fluids.

Principle of the Assay: The microtiter plate provided in this kit has been pre-coated with an antibody specific to NPC2. Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody preparation specific to NPC2. Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain NPC2, biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm +/- 10nm. The concentration of NPC2 in the samples is then determined by comparing the O.D. of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 5453678
• NCBI GeneID: 10577
• NCBI Accession #: NP_006423.1
• NCBI GenBank Nucleotide #: NM_006432.3
• UniProt Accession #: P61916; Q15668; Q29413; B4DQV7
• Molecular Weight: 13,416 Da
• NCBI Official Full Name: epididymal secretory protein E1
• NCBI Official Synonym Full Names: NPC intracellular cholesterol transporter 2
• NCBI Official Symbol: NPC2
• NCBI Official Synonym Symbols: HE1; EDDM1
• NCBI Protein Information: epididymal secretory protein E1
• UniProt Protein Name: Epididymal secretory protein E1
• Protein Family: Non-specific phospholipase
• UniProt Gene Name: NPC2
• UniProt Synonym Gene Names: HE1; He1

NCBI Description

This gene encodes a protein containing a lipid recognition domain. The encoded protein may function in regulating the transport of cholesterol through the late endosomal/lysosomal system. Mutations in this gene have been associated with Niemann-Pick disease, type C2 and frontal lobe atrophy. [provided by RefSeq, Jul 2008]

Uniprot Description

Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport.