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CLN3 Membrane Protein Introduction

Introduction of CLN3

CLN3 is encoded by the CLN3 gene, which is composed of 15 exons and encodes a transmembrane protein called CLN3P, Battenine or Batten disease protein. It belongs to the Neuronal ceroid lipofuscinoses family, which is a class of inherited progressive neurodegenerative diseases. CLN3 is a 438 amino acid transmembrane protein and its sequence is highly conserved among eukaryotes, suggesting CLN3 may play the same functions among all eukaryotes. Previous studies have reported that CLN3 is involved in mediating cell apoptosis in many neurodegenerative disorders.

Basic Information of CLN3
Protein Name Battenin
Gene Name CLN3
Aliases Batten disease protein, Protein CLN3
Organism Homo sapiens (Human)
UniProt ID Q13286
Transmembrane Times 6
Length (aa) 438
Sequence MGGCAGSRRRFSDSEGEETVPEPRLPLLDHQGAHWKNAVGFWLLGLCNNFSYVVMLSAAHDILSHKRTSGNQSHVDPGPTPIPHNSSSRFDCNSVSTAAVLLADILPTLVIKLLAPLGLHLLPYSPRVLVSGICAAGSFVLVAFSHSVGTSLCGVVFASISSGLGEVTFLSLTAFYPRAVISWWSSGTGGAGLLGALSYLGLTQAGLSPQQTLLSMLGIPALLLASYFLLLTSPEAQDPGGEEEAESAARQPLIRTEAPESKPGSSSSLSLRERWTVFKGLLWYIVPLVVVYFAEYFINQGLFELLFFWNTSLSHAQQYRWYQMLYQAGVFASRSSLRCCRIRFTWALALLQCLNLVFLLADVWFGFLPSIYLVFLIILYEGLLGGAAYVNTFHNIALETSDEHREFAMAATCISDTLGISLSGLLALPLHDFLCQLS

Function of CLN3 Membrane Protein

A variety of approaches have implicated that CLN3 plays important roles in many cellular biological processes, including intracellular protein trafficking, lysosomal homeostasis, and mitochondrial functions. The CLN3 protein is anti-apoptotic and it has previously been demonstrated that its integrity is necessary for neuronal and photoreceptor cell survival. Recently, it was reported that CLN3 genetically interacts with the oxidative stress signaling pathways in Drosophila. The lack of CLN3 function in cells leads to a failure of management in the response to ER stress and this may be the key deficit in e juvenile neuronal ceroid lipofuscinosis that causes neuronal degeneration. Moreover, CLN3 protecting cells from apoptosis after ER stress inducer tunicamycin treatment suggest that CLN3 directly interacts with ERS signaling pathway.

CLN3 Membrane Protein Introduction Fig.1 The signaling pathway of CLN3 in ER stress (Marotta, 2017).

Application of CLN3 Membrane Protein in Literature

  1. Wu D., et al. The Batten disease gene CLN3 confers resistance to endoplasmic reticulum stress induced by tunicamycin. Biochem. Biophys. Res. Commun. 2014, 447(1):115-120. PubMed ID: 24699413

    This article investigates the function of CLN3 in the ER stress signaling pathway. The results of this article suggest that the lack of CLN3 function in cells leads to a failure of management in the response to ER stress and this may be the key deficit in JNCL that causes neuronal degeneration.

  2. Makoukji J., et al. Association between CLN3 (Neuronal Ceroid Lipofuscinosis, CLN3 Type) Gene Expression and Clinical Characteristics of Breast Cancer Patients. Frontiers in Oncology. 2015, 5:215. PubMed ID: 26528430

    This article suggests that CLN3 may be a novel molecular target for cancer drug discovery with the goal of modulation of ceramide pathways because the relative overexpression of CLN3 mRNA transcripts in IDC breast FFPE and fresh tissues was significantly higher than in surrounding control non-tumor tissues or in control non-tumor tissue from reduction mammoplasties.

  3. Zhu X., et al. Effect of CLN3 silencing by RNA interference on the proliferation and apoptosis of human colorectal cancer cells. Biomedicine & Pharmacotherapy. 2014, 68(3):253-258. PubMed ID: 24556023

    The studies in this article demonstrate that CLN3 was expressed in colorectal cancer cells at a high frequency. Moreover, CLN3 down-regulation with RNA interference can inhibit proliferation, apoptosis, and cell cycle progression of colorectal cancer cells.

  4. Aldrich A., et al. Efficacy of phosphodiesterase-4 inhibitors in juvenile Batten disease (CLN3). Ann. Neurol. 2016, 80(6):909-923. PubMed ID: 27804148

    This article reports that cAMP levels were significantly reduced in the Cln3Δex7/8 brain, and were restored by PF-06266047. PDE4 inhibitors significantly improved motor function in Cln3Δex7/8 mice, attenuated glial activation and lysosomal pathology, and restored glutamate transporter expression to levels observed in WT animals, with no evidence of toxicity as revealed by blood chemistry analysis.

  5. Tecedor L., et al. CLN3 Loss Disturbs Membrane Microdomain Properties and Protein Transport in Brain Endothelial Cells. The Journal of Neuroscience. 2013, 33(46):18065-18079. PubMed ID: 24227717

    The authors in this article show that CLN3 localizes to the trans-Golgi network (TGN) and partitions with buoyant microdomain fractions. Moreover, the authors in this article propose that CLN3 facilitates TGN-to-plasma membrane transport of microdomain-associated proteins. Insult to this pathway may underlie BBB dysfunction and contribute to JNCL pathogenesis.

CLN3 Preparation Options

To obtain the soluble and functional target protein, the versatile Magic™ membrane protein production platform in Creative Biolabs enables many flexible options, from which you can always find a better match for your particular project. Besides, aided by our versatile Magic™ anti-membrane protein antibody discovery platform, we also provide customized anti-CLN3 antibody development services.


As a forward-looking research institute as well as a leading custom service provider in the field of membrane protein, Creative Biolabs is always providing the 100 points of the service for our worldwide customers to accomplish numerous challenging projects including generation of many functional membrane proteins. Please feel free to contact us for more information.

Reference

  1. Marotta D., et al. (2017) NCLs and ER: A stressful relationship. Biochimica et Biophysica Acta. 1863(6):1273-1281.

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