Anti-TPP1 Antibody, clone 2E12

产品说明

一般描述

Tripeptidyl-peptidase 1 (UniProt: O14773; also known as TPP1, Cell growth-inhibiting gene 1 protein, Lysosomal pepstatin-insensitive protease, LPIC, Tripeptidyl aminopeptidase) is encoded by the TPP1 (also known as CLN2, GIG1, UNQ267/PRO304) gene (Gene ID: 1200) in human. TPP1 is a lysosomal serine protease that acts as a non-specific peptidase and sequentially removes tripeptides from the free N termini of small polypeptides produced by lysosomal proteinases. TPP1 is expressed in several tissues, with tissues associated with peptide hormone production displaying higher level of expression. Human TPP I is synthesized as a zymogen with an apparent mass of 68 kDa, which is processed to a 48 kDa mature enzyme by removal of 179 amino acid prodomain. TPP I zymogen has five potential N-glycosylation sites at amino acid positions 210, 222, 286, 313, and 443. The N-linked glycans can add around 10 kDa to the molecular mass of TPP 1. Like many other lysosomal hydrolases, TPP I proenzyme is able to autoactivate in the acidic pH in vitro. Mutations in TPP1 gene are shown to be a causative factor in ceroid lipofuscinosis, neuronal, 2 (CLN2) a progressive neurodegenerative, lysosomal storage disease, characterized by intracellular accumulation of autofluorescent liposomal material and clinically manifested seizures, dementia, visual loss, and/or cerebral atrophy.

特异性

Clone 2E12 specifically immunostains tripeptidyl peptidase 1 in human placenta, heart, and pancreas.

免疫原

A recombinant fragment corresponding to amino acids 195-563 of human TPP1.

应用

Anti-TPP1, clone 2E12, Cat. No. MABN1806, is a highly specific mouse monoclonal antibody, that targets TPP1/Tripeptidyl-peptidase 1 and has been tested in Immunocytochemistry, Immunohistochemistry (Paraffin) and Western Blotting.
Immunohistochemistry Analysis: A 1:5,000 dilution from a representative lot detected TPP1 in human heart and human pancreas tissue.

Western Blotting Analysis: A 1:50 dilution from a representative lot detected TPP1 in CHO cells transfected with TPP1 and TPP1 mutant (Courtesy of Adam Golabek, Ph.D., New York State Institute for Basic Research in Developmental Disabilities, New York, USA).

Immunocytochemistry Analysis: A 1:50 dilution from a representative lot detected TPP1 in CHO cells expressing WT TPP1 (Courtesy of Adam Golabek, Ph.D., New York State Institute for Basic Research in Developmental Disabilities, New York, USA).





Research Category
Neuroscience

质量

Evaluated by Immunohistochemistry in human placenta tissue.

Immunohistochemistry Analysis: A 1:5,000 dilution of this antibody detected TPP1 in human placenta tissue.

目标描述

61.25 kDa calculated (composed of 6 identical subunits)

外形

Protein G purified
Format: Purified
Purified mouse monoclonal antibody IgG2b in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

储存及稳定性

Stable for 1 year at 2-8°C from date of receipt.

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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