CLN2 Antibody (D-11): sc-365838

CLN2 Antibody (D-11) is a mouse monoclonal IgG1 antibody that detects CLN2 in human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). CLN2, also known as Tripeptidyl peptidase I (TTP-I), is a vital lysosomal aminopeptidase that plays a significant role in the degradation of proteins within lysosomes, a process essential for cellular homeostasis and the recycling of amino acids. Deficiencies in CLN2 can lead to severe neurodegenerative diseases, highlighting its importance in maintaining neuronal health. CLN2 operates by sequentially removing tripeptides from small polypeptides, and exhibits minor endoprotease activity, which is essential for the processing of various substrates. In the lysosomal environment, the proenzyme form of CLN2 is converted into its active form with the help of another protease, and can autoactivate in acidic conditions through an unimolecular mechanism, ensuring effective function in the unique pH of lysosomes. Anti-CLN2 antibody (D-11) is an invaluable tool for researchers studying lysosomal function and related pathologies, providing insights into the molecular mechanisms underlying neurodegenerative diseases.

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CLN2 Antibody (D-11) References:

1. Neural and extraneural expression of the neuronal ceroid lipofuscinoses genes CLN1, CLN2, and CLN3: functional implications for CLN3.  |  Chattopadhyay, S. and Pearce, DA. 2000. Mol Genet Metab. 71: 207-11. PMID: 11001812
2. Neuronal ceroid lipofuscinoses caused by defects in soluble lysosomal enzymes (CLN1 and CLN2).  |  Hofmann, SL., et al. 2002. Curr Mol Med. 2: 423-37. PMID: 12125808
3. Neurofilament light is a treatment-responsive biomarker in CLN2 disease.  |  Ru, Y., et al. 2019. Ann Clin Transl Neurol. 6: 2437-2447. PMID: 31814335
4. Changing Times for CLN2 Disease: The Era of Enzyme Replacement Therapy.  |  Specchio, N., et al. 2020. Ther Clin Risk Manag. 16: 213-222. PMID: 32280231
5. MRI in CLN2 disease patients: Subtle features that support an early diagnosis.  |  Aydın, K., et al. 2020. Eur J Paediatr Neurol. 28: 228-236. PMID: 32855042
6. Managing CLN2 disease: a treatable neurodegenerative condition among other treatable early childhood epilepsies.  |  Mazurkiewicz-Bełdzińska, M., et al. 2021. Expert Rev Neurother. 21: 1275-1282. PMID: 33538188
7. Natural History Studies in NCL and Their Expanding Role in Drug Development: Experiences From CLN2 Disease and Relevance for Clinical Trials.  |  Nickel, M. and Schulz, A. 2022. Front Neurol. 13: 785841. PMID: 35211079
8. CLN2 disease resulting from a novel homozygous deep intronic splice variant in TPP1 discovered using long-read sequencing.  |  Steigerwald, C., et al. 2023. Mol Genet Metab. 140: 107713. PMID: 37922835
9. Safety and efficacy of cerliponase alfa in children with neuronal ceroid lipofuscinosis type 2 (CLN2 disease): an open-label extension study.  |  Schulz, A., et al. 2024. Lancet Neurol. 23: 60-70. PMID: 38101904
10. Evolution of Movement Disorders in Patients With CLN2-Batten Disease Treated With Enzyme Replacement Therapy.  |  Spaull, R., et al. 2024. Neurology. 103: e209615. PMID: 38976822

The preferred CLN2 antibody is CLN2 Antibody (G-3): sc-393961.