Human Epididymal secretory protein E1, NPC2 ELISA Kit | MyBioSource MBS909574 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Epididymal secretory protein E1, NPC2 ELISA Kit

catalog :

MBS909574

quantity :

48-Strip-Wells

price :

510 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS909574

products type :

ELISA Kit

products full name :

Human Epididymal secretory protein E1, NPC2 ELISA Kit

products short name :

Niemann-Pick disease, type C2

products name syn :

Human Epididymal secretory protein E1 (NPC2) ELISA kit; EDDM1; HE1; MGC1333; NP-C2; epididymal protein 1; epididymal secretory protein; epididymal secretory protein E1; tissue-specific secretory protein; Niemann-Pick disease; type C2

other names :

epididymal secretory protein E1; Epididymal secretory protein E1; epididymal secretory protein E1; epididymal protein 1; tissue-specific secretory protein; human epididymis-specific protein 1; Niemann-Pick disease type C2 protein; Niemann-Pick disease, type C2; Human epididymis-specific protein 1; He1; Niemann-Pick disease type C2 protein

products gene name :

NPC2

other gene names :

NPC2; NPC2; HE1; EDDM1; HE1; He1

uniprot entry name :

NPC2_HUMAN

reactivity :

Human

sequence length :

151

specificity :

This assay has high sensitivity and excellent specificity for detection of Human NPC2. No significant cross-reactivity or interference between Human NPC2 and analogues was observed.

storage stability :

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

other info1 :

Samples: Serum, plasma, tissue homogenates, Cell lysates. Assay Type: Sandwich. Detection Range: 0.312 ng/ml-20 ng/ml. Sensitivity: 0.078 ng/ml

other info2 :

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

products description :

Principle of the Assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for NPC2 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any NPC2 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for NPC2 is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of NPC2 bound in the initial step. The color development is stopped and the intensity of the color is measured.

ncbi gi num :

5453678

ncbi acc num :

NP_006423.1

ncbi gb acc num :

NM_006432.3

uniprot acc num :

P61916

ncbi mol weight :

16,570 Da

ncbi pathways :

Lysosome Pathway (99052); Lysosome Pathway (96865)

ncbi summary :

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 summary :

NPC2: 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. Defects in NPC2 are the cause of Niemann-Pick disease type C2 (NPDC2). A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C2 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood. Belongs to the NPC2 family. Protein type: Secreted, signal peptide; Secreted. Chromosomal Location of Human Ortholog: 14q24.3. Cellular Component: endoplasmic reticulum; lysosome. Molecular Function: protein binding; enzyme binding; cholesterol binding. Biological Process: cholesterol metabolic process; cholesterol homeostasis; cholesterol transport; intracellular sterol transport; regulation of isoprenoid metabolic process; response to virus; cholesterol efflux; intracellular cholesterol transport; phospholipid transport; glycolipid transport. Disease: Niemann-pick Disease, Type C2

size1 :

48-Strip-Wells

price1 :

510 USD

size2 :

96-Strip-Wells

price2 :

725

size3 :

5x96-Strip-Wells

price3 :

2565

size4 :

10x96-Strip-Wells

price4 :

4800

more info or order :

MyBioSource product webpage