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Journal articles on the topic 'Proprotein convertase 1 – génétique'

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Published: 7 July 2024

Last updated: 7 July 2024

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1

Kumar, Gurinder, Rajendran Nair, and Aman P. S. Sohal. "Proprotein Convertase 1/3 Deficiency." Indian Journal of Pediatrics 85, no. 4 (September 30, 2017): 320–21. http://dx.doi.org/10.1007/s12098-017-2479-x.

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2

Pasquato, Antonella, Philomena Pullikotil, Marie-Claude Asselin, Manuela Vacatello, Livio Paolillo, Francesca Ghezzo, Federica Basso, Carlo Di Bello, Monica Dettin, and Nabil G. Seidah. "The Proprotein Convertase SKI-1/S1P." Journal of Biological Chemistry 281, no. 33 (June 21, 2006): 23471–81. http://dx.doi.org/10.1074/jbc.m513675200.

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3

Pullikotil, Philomena, Suzanne Benjannet, Janice Mayne, and Nabil G. Seidah. "The Proprotein Convertase SKI-1/S1P." Journal of Biological Chemistry 282, no. 37 (July 10, 2007): 27402–13. http://dx.doi.org/10.1074/jbc.m703200200.

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4

Yana, Ikuo, and Stephen J. Weiss. "Regulation of Membrane Type-1 Matrix Metalloproteinase Activation by Proprotein Convertases." Molecular Biology of the Cell 11, no. 7 (July 2000): 2387–401. http://dx.doi.org/10.1091/mbc.11.7.2387.

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Membrane type-1 matrix metalloproteinase (MT1-MMP) is the prototypical member of a subgroup of membrane-anchored proteinases that belong to the matrix metalloproteinase family. Although synthesized as a zymogen, MT1-MMP plays an essential role in extracellular matrix remodeling after an undefined process that unmasks its catalytic domain. We now report the existence of a proprotein convertase–MT1-MMP axis that regulates the processing and functional activity of the metalloproteinase. Two sets of basic motifs in the propeptide region of MT1-MMP are identified that potentially can be recognized by the proprotein convertase family of subtilisin-like proteases. Processing of proMT1-MMP as well as the expression of its proteolytic activity were blocked by mutating these recognition motifs or by inhibiting the proprotein convertases furin and PC6 with the serpin-based inhibitor α1 antitrypsin Portland. Furthermore, both furin-dependent and furin-independent MT1-MMP processing pathways are identified that require tethering of the metalloproteinase to the cell surface. These findings demonstrate the existence of a proprotein convertase–MT1-MMP axis that can regulate extracellular matrix remodeling.

5

Icimoto, Marcelo Y., Nilana M. Barros, Juliana C. Ferreira, Marcelo F. Marcondes, Douglas Andrade, Mauricio F. Machado, Maria A. Juliano, Wagner A. Júdice, Luiz Juliano, and Vitor Oliveira. "Hysteretic Behavior of Proprotein Convertase 1/3 (PC1/3)." PLoS ONE 6, no. 9 (September 15, 2011): e24545. http://dx.doi.org/10.1371/journal.pone.0024545.

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6

Oral, H. "Proprotein Convertase 1/3 Deficiency with Pelvic Ewing Sarcoma." Acta Endocrinologica (Bucharest) 18, no. 4 (2022): 508–11. http://dx.doi.org/10.4183/aeb.2022.508.

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7

Jirholt, Pernilla, Martin Adiels, and Jan Borén. "How Does Mutant Proprotein Convertase Neural Apoptosis-Regulated Convertase 1 Induce Autosomal Dominant Hypercholesterolemia?" Arteriosclerosis, Thrombosis, and Vascular Biology 24, no. 8 (August 2004): 1334–36. http://dx.doi.org/10.1161/01.atv.0000133682.97348.ff.

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8

Seidah, N. G., S. Benjannet, L. Wickham, J. Marcinkiewicz, S. B. Jasmin, S. Stifani, A. Basak, A. Prat, and M. Chretien. "The secretory proprotein convertase neural apoptosis-regulated convertase 1 (NARC-1): Liver regeneration and neuronal differentiation." Proceedings of the National Academy of Sciences 100, no. 3 (January 27, 2003): 928–33. http://dx.doi.org/10.1073/pnas.0335507100.

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9

Lee, Y. C., A. B. Damholt, N. Billestrup, T. Kisbye, P. Galante, B. Michelsen, H. Kofod, and J. H. Nielsen. "Developmental expression of proprotein convertase 1/3 in the rat." Molecular and Cellular Endocrinology 155, no. 1-2 (September 1999): 27–35. http://dx.doi.org/10.1016/s0303-7207(99)00119-7.

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10

Ahmed, Amna Basheer M., and Badr M. Rasheed Alsaleem. "Enteroendocrine Dysfunction in Two Saudi Sisters." Case Reports in Gastroenterology 15, no. 1 (March 4, 2021): 290–95. http://dx.doi.org/10.1159/000511761.

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Proprotein convertase (PC) deficiency is a rare autosomal recessive disorder caused by mutations in proprotein convertase subtilisin/kexin type 1 (<i>PCSK1</i>). It is characterized by severe malabsorptive early-onset diarrhea, obesity, and systemic endocrinopathies. Only few cases have been reported in the literature; we have add two female sisters with some difference in clinical progress. Herein, we describe two sisters with congenital osmotic diarrhea diagnosed with PC1/3 deficiency, causing malabsorptive diarrhea and enteroendocrine dysfunction, who presented with chronic enteropathy with hypernatremia but with different expressivity. PC1/3 deficiency presents with symptoms and signs that mimic glucose-galactose malabsorption. Because of the clinical paucity and heterogeneity of congenital enteropathies, whole-exome sequencing may be of great help towards early diagnosis and effective treatment.

11

Tang, S. S., Z. Y. Liang, L. R. Guo, J. H. Zhang, and D. Zhou. "Proprotein convertase 1 mediated proneuropeptide proteolytic processing in ischemic neuron injury." Bratislava Medical Journal 118, no. 10 (2018): 609–12. http://dx.doi.org/10.4149/bll_2017_117.

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12

Rozanov, Dmitri V., and Alex Y. Strongin. "Membrane Type-1 Matrix Metalloproteinase Functions as a Proprotein Self-convertase." Journal of Biological Chemistry 278, no. 10 (January 3, 2003): 8257–60. http://dx.doi.org/10.1074/jbc.m213246200.

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13

Stein, Evan A. "Proprotein Convertase Subtilisin/kexin Type 9 Inhibitors in Clinical Practice: A Focused Update." US Cardiology Review 11, no. 2 (2017): 105. http://dx.doi.org/10.15420/usc.2017:23:1.

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This article provides an updated review of the LDL-cholesterol efficacy, safety, and cardiovascular benefits of proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors. It focuses on evidence from numerous clinical trials and provides clinicians with a basis for understanding, assessing, and selecting these agents for clinical practice. It also provides some perspective on other potential agents in development that target PCSK9.

14

Dubuc, Geneviève, Ann Chamberland, Hanny Wassef, Jean Davignon, Nabil G. Seidah, Lise Bernier, and Annik Prat. "Statins UpregulatePCSK9, the Gene Encoding the Proprotein Convertase Neural Apoptosis-Regulated Convertase-1 Implicated in Familial Hypercholesterolemia." Arteriosclerosis, Thrombosis, and Vascular Biology 24, no. 8 (August 2004): 1454–59. http://dx.doi.org/10.1161/01.atv.0000134621.14315.43.

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15

Bernot, D., J. Stalin, P. Stocker, B. Bonardo, I. Scroyen, M. C. Alessi, and F. Peiretti. "Plasminogen activator inhibitor 1 is an intracellular inhibitor of furin proprotein convertase." Journal of Cell Science 124, no. 8 (March 15, 2011): 1224–30. http://dx.doi.org/10.1242/jcs.079889.

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16

Basak, Ajoy, Peter Koch, Marcel Dupelle, Lloyd D. Fricker, Lakshmi A. Devi, Michel Chrétien, and Nabil G. Seidah. "Inhibitory Specificity and Potency of proSAAS-derived Peptides toward Proprotein Convertase 1." Journal of Biological Chemistry 276, no. 35 (July 2, 2001): 32720–28. http://dx.doi.org/10.1074/jbc.m104064200.

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17

Croissandeau, Gilles, Ajoy Basak, Nabil G. Seidah, Michel Chrétien, and Majambu Mbikay. "Proprotein convertases are important mediators of the adipocyte differentiation of mouse 3T3-L1 cells." Journal of Cell Science 115, no. 6 (March 15, 2002): 1203–11. http://dx.doi.org/10.1242/jcs.115.6.1203.

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Mouse 3T3-L1 cells are widely used to study adipocyte differentiation in vitro. When treated with insulin, dexamethasone and isobutylmethylxanthine these fibroblastic cells differentiate into round triglyceride-rich adipocytes. Because several proteins implicated in adipocyte differentiation(e.g. type 1 IGF receptors) are proteolytically activated by endoproteinases of the proprotein convertase family, we sought to determine whether these endoproteinases are crucial for adipose conversion. In this study, we show that expression of the proprotein convertases PACE4, PC7 and furin increases when 3T3-L1 cells are induced to differentiate into adipocytes. The differentiation was blocked in transfected cells expressingα1-antitrypsin Portland or in normal cells pre-treated with the synthetic inhibitor decanoyl-RVKR-chloromethylketone. Both inhibitors are known to specifically inactivate proprotein convertases. The block was associated with impaired proteolytic activation of proIGF-1 receptor, absence of induction of the adipogenic transcriptional factor PPARγ and marked reduction of the nuclear translocation of the C/EBPβ factor. Taken together, these data constitute evidence that proprotein convertases are crucial mediators of adipogenesis.

18

Gorski, Jeff P., Nichole T. Huffman, Chaoying Cui, Ellen P. Henderson, Ronald J. Midura, and Nabil G. Seidah. "Potential Role of Proprotein Convertase SKI-1 in the Mineralization of Primary Bone." Cells Tissues Organs 189, no. 1-4 (August 26, 2008): 25–32. http://dx.doi.org/10.1159/000151723.

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19

Lee, Sang-Nam, Da-Hyung Lee, Myung Hyun Sohn, and Joo-Heon Yoon. "Overexpressed proprotein convertase 1/3 induces an epithelial-mesenchymal transition in airway epithelium." European Respiratory Journal 42, no. 5 (January 11, 2013): 1379–90. http://dx.doi.org/10.1183/09031936.00100412.

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20

Duhamel, Marie, Franck Rodet, Adriana Murgoci, Maxence Wisztorski, Robert Day, Isabelle Fournier, and Michel Salzet. "Proprotein convertase 1/3 inhibited macrophages: A novel therapeutic based on drone macrophages." EuPA Open Proteomics 11 (June 2016): 20–22. http://dx.doi.org/10.1016/j.euprot.2016.03.003.

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21

Pullikotil, Philomena, Martin Vincent, Stuart T. Nichol, and Nabil G. Seidah. "Development of Protein-based Inhibitors of the Proprotein of Convertase SKI-1/S1P." Journal of Biological Chemistry 279, no. 17 (February 16, 2004): 17338–47. http://dx.doi.org/10.1074/jbc.m313764200.

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22

Jackson, Robert S., John W. M. Creemers, I. Sadaf Farooqi, Marie-Laure Raffin-Sanson, Andrea Varro, Graham J. Dockray, Jens J. Holst, et al. "Small-intestinal dysfunction accompanies the complex endocrinopathy of human proprotein convertase 1 deficiency." Journal of Clinical Investigation 112, no. 10 (November 15, 2003): 1550–60. http://dx.doi.org/10.1172/jci200318784.

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23

Salzet, Michel, Duhamel Marie, Rodet Franck, Wisztorski Maxence, and Fournier Isabelle. "Proprotein convertase 1/3 inhibited macrophages: A novel therapeutic based on drone macrophages." Journal of Biotechnology 231 (August 2016): S6. http://dx.doi.org/10.1016/j.jbiotec.2016.05.046.

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24

Li, Wei, Heegeun Park, Erling Guo, Wooyeon Jo, Kyu Min Sim, and Sang Ki Lee. "Aerobic Exercise Training Inhibits Neointimal Formation via Reduction of PCSK9 and LOX-1 in Atherosclerosis." Biomedicines 8, no. 4 (April 19, 2020): 92. http://dx.doi.org/10.3390/biomedicines8040092.

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The purpose of this study was to investigate whether aerobic exercise training inhibits atherosclerosis via the reduction of proprotein convertase subtilisin/kexin type 9 (PCSK9) expression in balloon-induced common carotid arteries of a high-fat-diet rats. Male SD (Sprague Dawley) rats fed an eight-weeks high-fat diet were randomly divided into three groups; these were the sham-operated control (SC), the balloon-induced control (BIC) and the balloon-induced exercise (BIE). The aerobic exercise training groups were performed on a treadmill. The major findings were as follows: first, body weight gain was significantly decreased by aerobic exercise training compared to the BIC without change of energy intake. Second, neointimal formation was significantly inhibited by aerobic exercise training in the balloon-induced common carotid arteries of high-fat-diet rats compared to the BIC. Third, low-density lipoprotein (LDL) receptor (LDLr) expression was significantly increased by aerobic exercise training in the livers of the high-fat diet group compared to the BIC, but not the proprotein convertase subtilisin/kexin type 9 (PCSK9) expression. Fourth, aerobic exercise training significantly decreased the expression of PCSK9, the lectin-like oxidized LDL receptor-1 (LOX-1), and vascular cell adhesion molecule-1 (VCAM-1) in balloon-induced common carotid arteries of high-fat-diet rats compared to the BIC. In conclusion, our results suggest that aerobic exercise training increases LDLr in the liver and inhibits neointimal formation via the reduction of PCSK9 and LOX-1 in balloon-induced common carotid arteries of high-fat-diet-induced rats.

25

HOSPITAL, Véronique, Valérie CHESNEAU, Agnès BALOGH, Catherine JOULIE, Nabil G. SEIDAH, Paul COHEN, and Annik PRAT. "N-arginine dibasic convertase (nardilysin) isoforms are soluble dibasic-specific metalloendopeptidases that localize in the cytoplasm and at the cell surface." Biochemical Journal 349, no. 2 (July 10, 2000): 587–97. http://dx.doi.org/10.1042/bj3490587.

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N-arginine (R) dibasic (NRD) convertase (nardilysin; EC 3.4.24.61), a metalloendopeptidase of the M16 family, specifically cleaves peptide substrates at the N-terminus of arginines in dibasic motifs in vitro. In rat testis, the enzyme localizes within the cytoplasm of spermatids and associates with microtubules of the manchette and axoneme. NRD1 and NRD2 convertases, two NRD convertase isoforms, differ by the absence (isoform 1) or presence (isoform 2) of a 68-amino acid insertion close to the active site. In this study, we overexpressed both isoforms, either by vaccinia virus infection of BSC40 cells or transfection of COS-7 cells. The partially purified enzymes exhibit very similar biochemical and enzymic properties. Microsequencing revealed that NRD convertase is N-terminally processed. Results of immunocytofluorescence, immunoelectron microscopy and subcellular fractionation studies argue in favour of a primary cytosolic localization of both peptidases. Although the putative signal peptide did not direct NRD convertase into microsomes in an in vitro translation assay, biotinylation experiments clearly showed the presence of both isoforms at the cell surface. In conclusion, although most known processing events at pairs of basic residues are achieved by proprotein convertases within the secretory pathway, NRD convertase may fulfil a similar function in the cytoplasm and/or at the cell surface.

26

Teitelman, Gladys. "Heterogeneous Expression of Proinsulin Processing Enzymes in Beta Cells of Non-diabetic and Type 2 Diabetic Humans." Journal of Histochemistry & Cytochemistry 67, no. 6 (February 13, 2019): 385–400. http://dx.doi.org/10.1369/0022155419831641.

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Although there is evidence indicating transcriptional and functional heterogeneity in human beta cells, it is unclear whether this heterogeneity extends to the expression level of the enzymes that process proinsulin to insulin in beta cells. To address this question, the expression levels of prohormone convertases (PC) 1/3, proprotein convertase 2 (PC2), and carboxypeptidase E (CPE) were determined in immune-stained sections of human pancreas. In non-diabetic donors, the level of proprotein convertase 1/3 (PC1/3) expression varied among beta cells of each islet but the average per islet was similar for all islets of each donor. Although the average PC1/3 expression of all islets examined per sample was unique for each pancreas, donors had similar levels of proinsulin/insulin expression. PC2 expression in beta cells showed less pronounced inter- and intraislet variation while CPE levels were fairly constant. The relationship between PC1/3 and PC2 expression levels was variable among different donors. Type 2 diabetes had an uneven effect on the expression levels of all three enzymes as they decrease only in some islets in a section. These findings suggest the presence of intraislet, but not interislet, variation in the expression of the proinsulin processing enzymes in non-diabetic subjects and a heterogeneous effect of type 2 diabetes on enzyme expression in islets.

27

Guillemot, Johann, Maryssa Canuel, Rachid Essalmani, Annik Prat, and Nabil G. Seidah. "Implication of the proprotein convertases in iron homeostasis: Proprotein convertase 7 sheds human transferrin receptor 1 and furin activates hepcidin." Hepatology 57, no. 6 (May 15, 2013): 2514–24. http://dx.doi.org/10.1002/hep.26297.

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28

Srour, Nadim, Annie Lebel, Stephanie McMahon, Isabelle Fournier, Martin Fugère, Robert Day, and Claire M. Dubois. "TACE/ADAM-17 maturation and activation of sheddase activity require proprotein convertase activity." FEBS Letters 554, no. 3 (October 17, 2003): 275–83. http://dx.doi.org/10.1016/s0014-5793(03)01159-1.

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29

Schettler, Volker J. J. "Therapeutische Apherese." Nephrologie aktuell 27, no. 08 (October 2023): 345–47. http://dx.doi.org/10.1055/a-2055-4775.

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Trotz neuer Entwicklungen Low-Density-Lipoprotein-Cholesterin (LDL-C) senkender Medikamente wie Statine, Ezetimib sowie Inhibitoren von Proprotein Convertase Subtilisin/Kexin type 9 (PCSK9) wie die PCSK9-Antikörper Alirocumab und Evolocumab oder die PCSK9-Antisense-Therapie mit Inclisiran, besteht weiterhin für bestimmte Patienten die Indikation zur Lipoproteinapherese (LA), wenn alle Lebensstil- und medikamentösen Maßnahmen ausgeschöpft wurden (Ultima-Ratio-Therapie) 1. Als Entscheidungsgrundlage für die Indikation zur LA gilt die Richtlinie Methoden vertragsärztliche Versorgung, Anlage I Nr. 1: Ambulante Durchführung der Apheresen als extrakorporales Hämotherapieverfahren, Rechtsgrundlage: § 135 Abs. 1 SGB V, Gültigkeit: seit 01.01.1991, zuletzt geändert zum: 06.03.2015.

30

Leighton, Mat, and Karl E. Kadler. "Paired Basic/Furin-like Proprotein Convertase Cleavage of Pro-BMP-1 in thetrans-Golgi Network." Journal of Biological Chemistry 278, no. 20 (March 11, 2003): 18478–84. http://dx.doi.org/10.1074/jbc.m213021200.

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31

McMahon, Stephanie, Francine Grondin, Patrick P. McDonald, Darren E. Richard, and Claire M. Dubois. "Hypoxia-enhanced Expression of the Proprotein Convertase Furin Is Mediated by Hypoxia-inducible Factor-1." Journal of Biological Chemistry 280, no. 8 (December 15, 2004): 6561–69. http://dx.doi.org/10.1074/jbc.m413248200.

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32

Poirier, Steve, Annik Prat, Edwige Marcinkiewicz, Joanne Paquin, Babykumari P. Chitramuthu, David Baranowski, Benoit Cadieux, Hugh P. J. Bennett, and Nabil G. Seidah. "Implication of the proprotein convertase NARC-1/PCSK9 in the development of the nervous system." Journal of Neurochemistry 98, no. 3 (August 2006): 838–50. http://dx.doi.org/10.1111/j.1471-4159.2006.03928.x.

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33

Pesu, Marko, Linda Muul, Yuka Kanno, and John J. O'Shea. "Proprotein convertase furin is preferentially expressed in T helper 1 cells and regulates interferon gamma." Blood 108, no. 3 (August 1, 2006): 983–85. http://dx.doi.org/10.1182/blood-2005-09-3824.

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Abstract Interleukin 12 (IL-12) is a major inducer of interferon gamma (IFN-γ) and the principal mediator of T helper 1 (Th1) differentiation. To identify IL-12–regulated genes, which might contribute to Th1 differentiation and IFNG regulation, we employed microarray analysis. Surprisingly, a ubiquitously expressed proprotein convertase (PC), furin, was one of the most consistently IL-12–induced genes in T cells, and among PCs was the only one regulated by this cytokine. Furin was preferentially expressed in differentiated Th1 cells in a Stat4-dependent manner. Expression of furin enhanced IFN-γ secretion, whereas inhibition of furin interfered with IFN-γ production. Thus, we conclude that IL-12 induction of furin might represent a new aspect of IFN-γ regulation and control of Th1 differentiation.

34

Wang, Ping, Micky Tortorella, Kristen England, Anne-Marie Malfait, Gary Thomas, Elizabeth C. Arner, and Duanqing Pei. "Proprotein Convertase Furin Interacts with and Cleaves Pro-ADAMTS4 (Aggrecanase-1) in thetrans-Golgi Network." Journal of Biological Chemistry 279, no. 15 (January 26, 2004): 15434–40. http://dx.doi.org/10.1074/jbc.m312797200.

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35

Germain, Carly St, Gilles Croissandeau, Janice Mayne, Jay M. Baltz, Michel Chrétien, and Majambu Mbikay. "Expression and transient nuclear translocation of proprotein convertase 1 (PC1) during mouse preimplantation embryonic development." Molecular Reproduction and Development 72, no. 4 (September 14, 2005): 483–93. http://dx.doi.org/10.1002/mrd.20271.

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36

Chen, Liang, Hao Peng, Bo-Lin Wang, Wen-Yuan Yu, Xiao-Hang Ding, Ming-Xin Gao, and Yang Yu. "Trends and hotspots in familial hypercholesterolemia: A bibliometric systematic review from 2002 to 2022." Medicine 102, no. 28 (July 14, 2023): e34247. http://dx.doi.org/10.1097/md.0000000000034247.

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Background: We visually assessed the research hotspots of familial hypercholesterolemia (FH) using bibliometrics and knowledge mapping in light of the research state and development trend of FH. Methods: We employed bibliometric tools, such as CiteSpace and the alluvial generator, to illustrate the scientific accomplishments on FH by extracting pertinent literature on FH from the Web of Science Core Collection database from January 1, 2002, to December 31, 2022. Results: A total of 4402 papers in total were selected for study; 29.2% of all articles globally were from the USA, followed by the Netherlands and England. The University of Amsterdam, University of Oslo, and University of Western Australia are the 3 institutions with the most publications in this area. Gerald F. Watts, Raul D. Santos, and John J. P. Kastelein wrote the majority of the pieces that were published. The New England Journal of Medicine, Circulation, and Atherosclerosis were the journals with the greatest number of papers in this field. Prevalence and genetic analysis of FH, proprotein convertase subtilisin/kexin 9 inhibitors, and inclisiran are current research hotspots for the condition. Future research in this area will be focused on gene therapy. Conclusions: FH research has shown shows a trend of ascending followed by leveling off. The prevalence and diagnosis of FH, proprotein convertase subtilisin/kexin 9 inhibitors, inclisiran, and gene therapy are current research hotspots. This report may serve as a reference for current research trends.

37

Prokop, Edyta Kinga, Paweł Piotr Jagodziński, and Stefan Grajek. "Genetics in familial hypercholesterolaemia – from genetic research to new guidelines." Journal of Medical Science 88, no. 3 (April 3, 2019): 192–94. http://dx.doi.org/10.20883/jms.245.

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Familial Hypercholesterolaemia (FH) is genetic disorder touching up to 1 to 250 people, increasing the risk of atherosclerotic cardiovascular disease risk and early death by 3–13 times. The majority of mutations are autosomal dominant among 3 genes related to cholesterole metabolism: LDL‑receptor (LDLR), apolipoprotein B (APOB) or proprotein convertase subtilisin/kexin type 9 (PCSK9). It comprises 60% of reported cases, which still is not at satisfactory level. This article summarizes new research in the field of FH and points out new therapeutic methods — PCSK9 inhibitors as advised in new European Society of Cardiology guidelines od dyslipidaemias.

38

Ojanen, Markus J. T., Hannu Turpeinen, Zuzet M. Cordova, Milka M. Hammarén, Sanna-Kaisa E. Harjula, Mataleena Parikka, Mika Rämet, and Marko Pesu. "The Proprotein Convertase Subtilisin/Kexin FurinA Regulates Zebrafish Host Response against Mycobacterium marinum." Infection and Immunity 83, no. 4 (January 26, 2015): 1431–42. http://dx.doi.org/10.1128/iai.03135-14.

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Tuberculosis is a chronic bacterial disease with a complex pathogenesis. An effective immunity againstMycobacterium tuberculosisrequires both the innate and adaptive immune responses, including proper T helper (Th) type 1 cell function. FURIN is a proprotein convertase subtilisin/kexin (PCSK) enzyme, which is highly expressed in Th1 type cells.FURINexpression in T cells is essential for maintaining peripheral immune tolerance, but its role in the innate immunity and infections has remained elusive. Here, we utilizedMycobacterium marinuminfection models in zebrafish (Danio rerio) to investigate howfurinregulates host responses against mycobacteria. In steady-statefurinAtd204e/+fish reducedfurinAmRNA levels associated with low granulocyte counts and elevated Th cell transcription factor expressions. Silencingfuringenes reduced the survival ofM. marinum-infected zebrafish embryos. A mycobacterial infection upregulatedfurinAin adult zebrafish, and infectedfurinAtd204e/+mutants exhibited a proinflammatory phenotype characterized by elevatedtumor necrosis factor a(tnfa),lymphotoxin alpha(lta) andinterleukin 17a/f3(il17a/f3) expression levels. The enhanced innate immune response in thefurinAtd204e/+mutants correlated with a significantly decreased bacterial burden in a chronicM. marinuminfection model. Our data show that upregulatedfurinAexpression can serve as a marker for mycobacterial disease, since it inhibits early host responses and consequently promotes bacterial growth in a chronic infection.

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YOSHIDA, Ichiro, Shizuyo KOIDE, Shin-ichi HASEGAWA, Akira NAKAGAWARA, Akihiko TSUJI, and Yoshiko MATSUDA. "Proprotein convertase PACE4 is down-regulated by the basic helix–loop–helix transcription factor hASH-1 and MASH-1." Biochemical Journal 360, no. 3 (December 10, 2001): 683–89. http://dx.doi.org/10.1042/bj3600683.

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PACE4 is a mammalian subtilisin-like proprotein convertase that activates transforming growth factor (TGF)-β-related proteins such as bone morphogenetic protein 2 (BMP2), BMP4 and Nodal and exhibits a dynamic expression pattern during embryogenesis. We recently determined that the 1kb 5′-upstream region of the PACE4 gene contains 12 E-box (E1–E12) elements and that an E-box cluster (E4–E9) acts as a negative regulator [Tsuji, Yoshida, Hasegawa, Bando, Yoshida, Koide, Mori and Matsuda (1999) J. Biochem. (Tokyo) 126, 494–502]. It is known that the mammalian achaete–scute homologue 1 (MASH-1) binds specifically to an E-box (CACCTG) sequence in collaboration with E47, a ubiquitously expressed basic helix–loop–helix (bHLH) factor. To identify the roles of the bHLH factor and E-box elements in regulating PACE4 gene expression in neural development, we analysed the effects of human achaete–scute homologue 1 (hASH-1) on PACE4 gene expression with various neuroblastoma cell lines. The expressions of PACE4 and hASH-1 are correlated inversely in these cell lines. The overexpression of hASH-1 or MASH-1 causes a marked decrease in endogenous PACE4 gene expression but has no effect on the expression of other subtilisin-like proprotein convertases such as furin, PC5/6 and PC7/8. In contrast, other neural bHLH factors (MATH-1, MATH-2, neurogenin 1, neurogenin 2, neurogenin 3 and E47) did not affect PACE4 gene expression. Furthermore, an E-box cluster was a negative regulatory element for the promoter activity in NBL-S cells expressing hASH-1 at high level as determined by a luciferase assay. Binding of hASH-1 to the E-box cluster was confirmed by gel mobility-shift assay. In the present study we identified the PACE4 gene as one of the targets of hASH-1, which is a key factor in the initiation of neural differentiation. These results suggest that the alteration of PACE4 gene expression by hASH-1 causes rapid changes in the biological activities of TGF-β-related proteins via post-translational modification of these proteins.

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Awan, Zuhier, Alexis Baass, and Jacques Genest. "Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9): Lessons Learned from Patients with Hypercholesterolemia." Clinical Chemistry 60, no. 11 (November 1, 2014): 1380–89. http://dx.doi.org/10.1373/clinchem.2014.225946.

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BACKGROUND Identification of the proprotein convertase subtilisin/kexin type 9 (PCSK9) as the third gene causing familial hypercholesterolemia (FH) and understanding its complex biology has led to the discovery of a novel class of therapeutic agents. CONTENT PCSK9 undergoes autocatalytic cleavage in the endoplasmic reticulum and enters the secretory pathway. The PCSK9 gene is under the regulatory control of sterol receptor binding proteins 1 and 2. Statins increase PCSK9 and this may modulate the response to this class of medications. In plasma, PCSK9 binds to the epidermal growth factor–like domain of the LDL receptor (LDL-R) on the cell and, once incorporated in the late endosomal pathway, directs the LDL-R toward lysosomal degradation rather than recycling to the plasma membrane. Thus, gain-of-function PCSK9 mutations lead to an FH phenotype, whereas loss-of-function mutations are associated with increased LDL-R–mediated endocytosis of LDL particles and lower LDL cholesterol in plasma. Inhibition of PCSK9 is thus an attractive therapeutic target. Presently, this is achieved by using monoclonal antibodies for allosteric inhibition of the PCSK9–LDL-R interaction. Phase 2 and 3 clinical trials in patients with moderate and severe hypercholesterolemia (including FH) show that this approach is safe and highly efficacious to lower LDL-C and lipoprotein(a). SUMMARY PCSK9 has other biological roles observed in vitro and in animal studies, including viral entry into the cell, insulin resistance, and hepatic tissue repair. Given the potential number of humans exposed to this novel class of medications, careful evaluation of clinical trial results is warranted.

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Pasquato, Antonella, Dominique J. Burri, Esther Gomez-Ibarlucea Traba, Layane Hanna-El-Daher, Nabil G. Seidah, and Stefan Kunz. "Arenavirus envelope glycoproteins mimic autoprocessing sites of the cellular proprotein convertase subtilisin kexin isozyme-1/site-1 protease." Virology 417, no. 1 (August 2011): 18–26. http://dx.doi.org/10.1016/j.virol.2011.04.021.

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Gorski, Jeff P., Nichole T. Huffman, Sridar Chittur, Ronald J. Midura, Claudine Black, Julie Oxford, and Nabil G. Seidah. "Inhibition of Proprotein Convertase SKI-1 Blocks Transcription of Key Extracellular Matrix Genes Regulating Osteoblastic Mineralization." Journal of Biological Chemistry 286, no. 3 (November 12, 2010): 1836–49. http://dx.doi.org/10.1074/jbc.m110.151647.

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Mayer, Ga�tan, Guy Boileau, and Mo�se Bendayan. "The proprotein convertase furin colocalizes with caveolin-1 in the Golgi apparatus and endosomes of hepatocytes." Cell and Tissue Research 316, no. 1 (April 1, 2004): 55–63. http://dx.doi.org/10.1007/s00441-004-0866-x.

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Martín, Martín G., Iris Lindberg, R. Sergio Solorzano–Vargas, Jiafang Wang, Yaron Avitzur, Robert Bandsma, Christiane Sokollik, et al. "Congenital Proprotein Convertase 1/3 Deficiency Causes Malabsorptive Diarrhea and Other Endocrinopathies in a Pediatric Cohort." Gastroenterology 145, no. 1 (July 2013): 138–48. http://dx.doi.org/10.1053/j.gastro.2013.03.048.

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Yu, Fei, Jiehua Xu, Hongxun Chen, Siyang Song, Chunlan Nie, Kai Hao, and Zhe Zhao. "Proprotein convertase cleavage of Ictalurid herpesvirus 1 spike-like protein ORF46 is modulated by N-glycosylation." Virology 592 (April 2024): 110008. http://dx.doi.org/10.1016/j.virol.2024.110008.

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Li, Yongjun, Mingming Yang, Xi Chen, Rui Zhang, Jing Li, Xiaoguo Zhang, Pengfei Zuo, and Genshan Ma. "Effects of PCSK9 Inhibition on Coronary Atherosclerosis Regression of Nontarget Lesions after Primary Percutaneous Coronary Intervention in Acute Coronary Syndrome Patients." Journal of Interventional Cardiology 2022 (December 26, 2022): 1–5. http://dx.doi.org/10.1155/2022/4797529.

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Aims. To evaluate the regression of coronary atherosclerosis with proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibition in acute coronary syndrome (ACS) patients following primary percutaneous coronary intervention (PPCI). Methods and Result. We examined 40 nontarget lesions in 17 ACS patients who underwent PPCI and were treated with PCSK9 inhibitors. At 1 year, total cholesterol, low-density lipoprotein cholesterol (LDL-C), and atherogenic index (AI) decreased significantly by 2.5 mmol/L, 2.01 mmol/L, and 1.86, respectively. On quantitative coronary angiography, treatment with PCSK9 inhibitors reduced significantly the atherosclerotic area stenosis in nontarget lesions (61.18 ± 14.55 at baseline vs. 52.85 ± 15.51 at 1 year, P < 0.001). Conclusions. After 1 year of PCSK9 inhibition treatment for ACS patients, the area stenosis of non-TLR was considerably reduced.

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Jang, Hyun-Duk, Sang Eun Lee, Jimin Yang, Hyun-Chae Lee, Dasom Shin, Hwan Lee, Jaewon Lee, et al. "Cyclase-associated protein 1 is a binding partner of proprotein convertase subtilisin/kexin type-9 and is required for the degradation of low-density lipoprotein receptors by proprotein convertase subtilisin/kexin type-9." European Heart Journal 41, no. 2 (August 16, 2019): 239–52. http://dx.doi.org/10.1093/eurheartj/ehz566.

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Abstract Aims Proprotein convertase subtilisin/kexin type-9 (PCSK9), a molecular determinant of low-density lipoprotein (LDL) receptor (LDLR) fate, has emerged as a promising therapeutic target for atherosclerotic cardiovascular diseases. However, the precise mechanism by which PCSK9 regulates the internalization and lysosomal degradation of LDLR is unknown. Recently, we identified adenylyl cyclase-associated protein 1 (CAP1) as a receptor for human resistin whose globular C-terminus is structurally similar to the C-terminal cysteine-rich domain (CRD) of PCSK9. Herein, we investigated the role of CAP1 in PCSK9-mediated lysosomal degradation of LDLR and plasma LDL cholesterol (LDL-C) levels. Methods and results The direct binding between PCSK9 and CAP1 was confirmed by immunoprecipitation assay, far-western blot, biomolecular fluorescence complementation, and surface plasmon resonance assay. Fine mapping revealed that the CRD of PCSK9 binds with the Src homology 3 binding domain (SH3BD) of CAP1. Two loss-of-function polymorphisms found in human PCSK9 (S668R and G670E in CRD) were attributed to a defective interaction with CAP1. siRNA against CAP1 reduced the PCSK9-mediated degradation of LDLR in vitro. We generated CAP1 knock-out mice and found that the viable heterozygous CAP1 knock-out mice had higher protein levels of LDLR and lower LDL-C levels in the liver and plasma, respectively, than the control mice. Mechanistic analysis revealed that PCSK9-induced endocytosis and lysosomal degradation of LDLR were mediated by caveolin but not by clathrin, and they were dependent on binding between CAP1 and caveolin-1. Conclusion We identified CAP1 as a new binding partner of PCSK9 and a key mediator of caveolae-dependent endocytosis and lysosomal degradation of LDLR.

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Rojek, Jillian M., Andrew M. Lee, NgocThao Nguyen, Christina F. Spiropoulou, and Stefan Kunz. "Site 1 Protease Is Required for Proteolytic Processing of the Glycoproteins of the South American Hemorrhagic Fever Viruses Junin, Machupo, and Guanarito." Journal of Virology 82, no. 12 (April 9, 2008): 6045–51. http://dx.doi.org/10.1128/jvi.02392-07.

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ABSTRACT The cellular proprotein convertase site 1 protease (S1P) has been implicated in the proteolytic processing of the glycoproteins (GPs) of Old World arenaviruses. Here we report that S1P is also involved in the processing of the GPs of the genetically more-distant South American hemorrhagic fever viruses Guanarito, Machupo, and Junin. Efficient cleavage of Guanarito virus GP, whose protease recognition sites deviate from the reported S1P consensus sequence, indicates a broader specificity of S1P than anticipated. Lack of GP processing of Junin virus dramatically reduced production of infectious virus and prevented cell-to-cell propagation. Infection of S1P-deficient cells resulted in viral persistence over several weeks without the emergence of escape variants able to use other cellular proteases for GP processing.

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Roebroek, A. J., L. Umans, I. G. Pauli, E. J. Robertson, F. van Leuven, W. J. Van de Ven, and D. B. Constam. "Failure of ventral closure and axial rotation in embryos lacking the proprotein convertase Furin." Development 125, no. 24 (December 15, 1998): 4863–76. http://dx.doi.org/10.1242/dev.125.24.4863.

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We have examined the role of Furin in postimplantation-stage mouse embryos by analyzing both the expression pattern of fur mRNA and the developmental consequences of a loss-of-function mutation at the fur locus. At early stages (day 7.5), fur mRNA is abundant in extraembryonic endoderm and mesoderm, anterior visceral endoderm, and in precardiac mesoderm. 1 day later fur is expressed throughout the heart tube and in the lateral plate mesoderm, notochordal plate and definitive gut endoderm. Embryos lacking Furin die between days 10.5 and 11.5, presumably due to hemodynamic insufficiency associated with severe ventral closure defects and the failure of the heart tube to fuse and undergo looping morphogenesis. Morphogenesis of the yolk sac vasculature is also abnormal, although blood islands and endothelial precursors form. Analysis of cardiac and endodermal marker genes shows that while both myocardial precursors and definitive endoderm cells are specified, their numbers and migratory properties are compromised. Notably, mutant embryos fail to undergo axial rotation, even though Nodal and eHand, two molecular markers of left-right asymmetry, are appropriately expressed. Overall, the present data identify Furin as an important activator of signals responsible for ventral closure and embryonic turning.

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Hardman, B. M., L. M. Kilpatrick, A. N. Stephens, J. I. C. Chen, P. Stanton, L. A. Salamonsen, and G. Nie. "211. Proteomic identification of caldesmon as one of the physiological substrates of proprotein convertase 6 during decidualisation." Reproduction, Fertility and Development 20, no. 9 (2008): 11. http://dx.doi.org/10.1071/srb08abs211.

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We have previously demonstrated that proprotein convertase 5/6 (PC6), a member of the proprotein convertase (PC) family, is a critical endometrial factor for implantation. PC6 is upregulated in the endometrium specifically at implantation in association with epithelial differentiation (in human and monkey) and stromal cell decidualisation (in the mouse, human and monkey). Knockdown of endometrial PC6 during early pregnancy in mice in vivo led to complete failure of implantation, while blocking of PC6 production in human endometrial stromal cells in vitro inhibited decidualisation. PCs convert a range of precursor proteins of important functions into their bioactive forms; they are thus regarded as critical ‘master switch’ molecules. We hypothesise that PC6 exerts its roles in the endometrium by regulating proteins of diverse functions essential for implantation. In this study, we utilised proteomic technology and aimed to identify proteins that are specifically cleaved by PC6 in human endometrial stromal cells (HESC) during decidualisation. HESC were decidualised with cyclic AMP, the cell lysates were treated with and without recombinant human PC6-A (rPC6-A), and the 2D Differential in Gel Electrophoresis (2D DiGE) protein profiles were compared between the two treatments. We identified several proteins which were differentially cleaved following the addition of rPC6-A. Mass spectrometric analysis confirmed that the most abundant of these were caldesmon, tropomyosin-2, tropomyosin-4, hypoxia Inducible factor-1 and chloride intracellular channel-1. These proteins showed spot shifts in hPC6-A treated HESC lysates consistent with hPC6-A cleavage. western blot analysis confirmed the specific cleavage of caldesmon by PC6 in HESCs, and immunohistochemical analysis showed co-localisation of caldesmon and PC6 in decidual cells in human endometrial tissue. Given that caldesmon is a structural protein previously found to be involved in actin filament reorganisation, our results strongly suggest that PC6 is a mediator of structural remodelling of stromal cells during decidualisation in the endometrium.