catalog number :
MBS2500919
products type :
ELISA Kit
products full name :
Human TPP1 (Tripeptidyl Peptidase I) ELISA Kit
products short name :
[TPP1]
other names :
[tripeptidyl-peptidase 1 preproprotein; Tripeptidyl-peptidase 1; tripeptidyl-peptidase 1; cell growth-inhibiting gene 1 protein; growth-inhibiting protein 1; lysosomal pepstatin insensitive protease; tripeptidyl aminopeptidase; 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; TPP-1; LPIC; TPP-I]
uniprot entry name :
TPP1_HUMAN
specificity :
This kit recognizes Human TNNI1 in samples. No significant cross-reactivity or interference between HumanTNNI1and analogues was observed.
storage stability :
Store at 4 degree C.
image1 heading :
Typical Testing Data/Standard Curve (for reference only)
other info1 :
Samples: Serum, Plasma And Other Biological Fluids. Assay Type: Quantitative Sandwich. Detection Range: 46.88-3000pg/mL. Sensitivity: 28.13pg/mL.
products description :
Intended Uses: This ELISA kit applies to the in vitro quantitative determination of Human TNNI1 concentrations in serum, plasma and other biological fluids. Principle of the Assay: This ELISA kit uses the Sandwich-ELISA principle. The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to Human TNNI1. Standards or samples are added to the micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibody specific for Human TNNI1 and Avidin-Horseradish Peroxidase (HRP) conjugate are added successively to each micro plate well and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain Human TNNI1, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction is terminated by the addition of stop solution and the color turns yellow. The optical density (OD) is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of Human TNNI1. You can calculate the concentration of Human TNNI1 in the samples by comparing the OD of the samples to the standard curve.
ncbi acc num :
NP_000382.3
ncbi gb acc num :
NM_000391.3
ncbi mol weight :
34,464 Da
ncbi pathways :
IRE1alpha Activates Chaperones Pathway (105906); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Of Proteins Pathway (106230); Unfolded Protein Response (UPR) Pathway (105904); XBP1(S) Activates Chaperone Genes Pathway (530771)
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; EC 3.4.14.9; Protease; Mitochondrial; Secreted. 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; serine-type endopeptidase activity; metal ion binding; 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; neuromuscular process controlling balance; proteolysis; bone resorption. Disease: Ceroid Lipofuscinosis, Neuronal, 2
size5 :
10x96-Strip-Wells