catalog number :
MBS912101
products type :
ELISA Kit
products full name :
Human Tripeptidyl-peptidase 1, TPP1 ELISA Kit
products short name :
tripeptidyl peptidase I
products name syn :
Human Tripeptidyl-peptidase 1 (TPP1) ELISA kit; CLN2; GIG1; LPIC; MGC21297; ceroid-lipofuscinosis; neuronal 2; late infantile (Jansky-Bielschowsky disease) ; growth-inhibiting protein 1; lysosomal pepstatin insensitive protease; tripeptidyl aminopept; tripeptidyl peptidase I
other names :
tripeptidyl-peptidase 1 preproprotein; Tripeptidyl-peptidase 1; tripeptidyl-peptidase 1; tripeptidyl aminopeptidase; growth-inhibiting protein 1; cell growth-inhibiting gene 1 protein; lysosomal pepstatin insensitive protease; tripeptidyl peptidase I; Cell growth-inhibiting gene 1 protein; Lysosomal pepstatin-insensitive protease; LPIC; Tripeptidyl aminopeptidase; Tripeptidyl-peptidase I; TPP-I
products gene name :
TPP1
other gene names :
TPP1; TPP1; CLN2; GIG1; LPIC; SCAR7; TPP-1; CLN2; GIG1; UNQ267/PRO304; TPP-1; LPIC; TPP-I
uniprot entry name :
TPP1_HUMAN
specificity :
This assay has high sensitivity and excellent specificity for detection of Human TPP1. No significant cross-reactivity or interference between Human TPP1 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 and Cell lysates. Assay Type: Sandwich. Detection Range: 12.5 pg/ml-800 pg/ml. Sensitivity: 3.12 pg/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 TPP1 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any TPP1 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for TPP1 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 TPP1 bound in the initial step. The color development is stopped and the intensity of the color is measured.
ncbi acc num :
NP_000382.3
ncbi gb acc num :
NM_000391.3
ncbi mol weight :
61,248 Da
ncbi pathways :
Activation Of Chaperone Genes By XBP1(S) Pathway (530771); Activation Of Chaperones By IRE1alpha Pathway (105906); Diabetes Pathways (105902); Disease Pathway (530764); Lysosome Pathway (99052); Lysosome Pathway (96865); Unfolded Protein Response Pathway (105904)
ncbi summary :
This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. [provided by RefSeq, Jul 2008]
uniprot summary :
TPP1: Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus. Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2). A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN2 consists of curvilinear profiles. Belongs to the peptidase S53 family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Secreted, signal peptide; Protease; Secreted; Mitochondrial; EC 3.4.14.9. Chromosomal Location of Human Ortholog: 11p15. Cellular Component: lysosomal lumen; mitochondrion; lysosome; melanosome. Molecular Function: tripeptidyl-peptidase activity; peptidase activity; protein binding; serine-type peptidase activity; metal ion binding; serine-type endopeptidase activity; endopeptidase activity; peptide binding. Biological Process: nervous system development; cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; epithelial cell differentiation; lysosome organization and biogenesis; unfolded protein response; peptide catabolic process; protein catabolic process; lipid metabolic process; proteolysis; neuromuscular process controlling balance; bone resorption. Disease: Ceroid Lipofuscinosis, Neuronal, 2
size4 :
10x96-Strip-Wells