Product Name
CLN2 (TPP1), Polyclonal Antibody
Full Product Name
Anti-CLN2 Antibody
Product Synonym Names
CLN2; Tripeptidyl-peptidase 1; TPP-1; Cell growth-inhibiting gene 1 protein; Lysosomal pepstatin-insensitive protease; LPIC; Tripeptidyl aminopeptidase; Tripeptidyl-peptidase I; TPP-I
Product Gene Name
anti-TPP1 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for O14773
Species Reactivity
Human, Mouse, Rat
Specificity
Recognizes endogenous levels of CLN2 protein.
Purity/Purification
The antibody was purified by immunogen affinity chromatography.
Form/Format
Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.
Immunogen
Recombinant full length protein of human CLN2
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.
Other Notes
Small volumes of anti-TPP1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-TPP1 antibody
Rabbit polyclonal antibody to CLN2
Applications Tested/Suitable for anti-TPP1 antibody
Western Blot (WB)
Application Notes for anti-TPP1 antibody
Western Blot: 1/500 - 1/2000
Western Blot (WB) of anti-TPP1 antibody
Western blot analysis of CLN2 expression in SW480 (A), SKOV3 (B), HepG2 (C), mouse ovary (D) whole cell lysates.
NCBI/Uniprot data below describe general gene information for TPP1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000382.3
[Other Products]
NCBI GenBank Nucleotide #
NM_000391.3
[Other Products]
UniProt Primary Accession #
O14773
[Other Products]
UniProt Secondary Accession #
Q53HT1; Q5JAK6; Q6UX56; Q71JP6; Q96C37[Other Products]
UniProt Related Accession #
O14773[Other Products]
Molecular Weight
34,464 Da
NCBI Official Full Name
tripeptidyl-peptidase 1 preproprotein
NCBI Official Synonym Full Names
tripeptidyl peptidase 1
NCBI Official Symbol
TPP1 [Similar Products]
NCBI Official Synonym Symbols
CLN2; GIG1; LPIC; SCAR7; TPP-1
[Similar Products]
NCBI Protein Information
tripeptidyl-peptidase 1
UniProt Protein Name
Tripeptidyl-peptidase 1
UniProt Synonym Protein Names
Cell growth-inhibiting gene 1 protein; Lysosomal pepstatin-insensitive protease; LPIC; Tripeptidyl aminopeptidase; Tripeptidyl-peptidase I; TPP-I
Protein Family
Tripeptidyl-peptidase
UniProt Gene Name
TPP1 [Similar Products]
UniProt Synonym Gene Names
CLN2; TPP-1; LPIC; TPP-I [Similar Products]
UniProt Entry Name
TPP1_HUMAN
NCBI Summary for TPP1
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 Comments for TPP1
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: Mitochondrial; EC 3.4.14.9; Protease; Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 11p15
Cellular Component: lysosomal lumen; lysosome
Molecular Function: endopeptidase activity; peptidase activity; peptide binding; protein binding; serine-type peptidase activity; tripeptidyl-peptidase activity
Biological Process: bone resorption; central nervous system development; epithelial cell differentiation; lipid metabolic process; lysosome organization and biogenesis; nervous system development; neuromuscular process controlling balance; peptide catabolic process; proteolysis
Disease: Ceroid Lipofuscinosis, Neuronal, 2
Research Articles on TPP1
1. The conservation between fission yeast Tpz1-Pot1 and human TPP1-POT1 interactions resulted in mapping a human melanoma-associated POT1 mutation (A532P) to the TPP1-POT1 interface.
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
