Search Results - "Creemers, John W.M"

Impaired Islet Function in Commonly Used Transgenic Mouse Lines due to Human Growth Hormone Minigene Expression by Brouwers, Bas, de Faudeur, Geoffroy, Osipovich, Anna B., Goyvaerts, Lotte, Lemaire, Katleen, Boesmans, Leen, Cauwelier, Elisa J.G., Granvik, Mikaela, Pruniau, Vincent P.E.G., Van Lommel, Leentje, Van Schoors, Jolien, Stancill, Jennifer S., Smolders, Ilse, Goffin, Vincent, Binart, Nadine, in’t Veld, Peter, Declercq, Jeroen, Magnuson, Mark A., Creemers, John W.M., Schuit, Frans, Schraenen, Anica

“…The human growth hormone (hGH) minigene is frequently used in the derivation of transgenic mouse lines to enhance transgene expression. Although this minigene…”
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Proprotein convertases in human atherosclerotic plaques: The overexpression of FURIN and its substrate cytokines BAFF and APRIL by Turpeinen, Hannu, Raitoharju, Emma, Oksanen, Anna, Oksala, Niku, Levula, Mari, Lyytikäinen, Leo-Pekka, Järvinen, Otso, Creemers, John W.M, Kähönen, Mika, Laaksonen, Reijo, Pelto-Huikko, Markku, Lehtimäki, Terho, Pesu, Marko

“…Abstract Background Proprotein convertase subtilisin/kexin (PCSK) enzymes cleave proproteins into mature end products. Previously, MBTPS1 and PCSK9 have been…”
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Haploinsufficiency of the autism candidate gene Neurobeachin induces autism-like behaviors and affects cellular and molecular processes of synaptic plasticity in mice by Nuytens, Kim, Gantois, Ilse, Stijnen, Pieter, Iscru, Emilia, Laeremans, Annelies, Serneels, Lutgarde, Van Eylen, Lien, Liebhaber, Stephen A, Devriendt, Koen, Balschun, Detlef, Arckens, Lutgarde, Creemers, John W.M, D'Hooge, Rudi

“…Abstract Neurobeachin (NBEA), a brain-enriched multidomain scaffolding protein involved in neurotransmitter release and synaptic functioning, has been…”
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Functional and clinical relevance of novel and known PCSK1 variants for childhood obesity and glucose metabolism by Löffler, Dennis, Behrendt, Susanne, Creemers, John W.M, Klammt, Jürgen, Aust, Gabriela, Stanik, Juraj, Kiess, Wieland, Kovacs, Peter, Körner, Antje

“…Abstract Objective Variants in Proprotein Convertase Subtilisin/Kexin Type 1 ( PCSK1 ) may be causative for obesity as suggested by monogenic cases and…”
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PREPL, a prolyl endopeptidase-like enzyme by name only?--Lessons from patients by Boonen, Kurt, Régal, Luc, Jaeken, Jaak, Creemers, John W M

“…Deletion of the Prolyl Endopeptidase-like (PREPL) gene has been described in three contiguous gene deletion syndromes at the 2p21 locus and current…”
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Proprotein convertase furin regulates osteocalcin and bone endocrine function by Al Rifai, Omar, Chow, Jacqueline, Lacombe, Julie, Julien, Catherine, Faubert, Denis, Susan-Resiga, Delia, Essalmani, Rachid, Creemers, John Wm, Seidah, Nabil G, Ferron, Mathieu

“…Osteocalcin (OCN) is an osteoblast-derived hormone that increases energy expenditure, insulin sensitivity, insulin secretion, and glucose tolerance. The cDNA…”
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PREPL deficiency with or without cystinuria causes a novel myasthenic syndrome by Régal, Luc, Shen, Xin-Ming, Selcen, Duygu, Verhille, Chantal, Meulemans, Sandra, Creemers, John W.M, Engel, Andrew G

“…OBJECTIVE:To investigate the genetic and physiologic basis of the neuromuscular symptoms of hypotonia-cystinuria syndrome (HCS) and isolated PREPL deficiency,…”
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PREPL deficiency: delineation of the phenotype and development of a functional blood assay by Régal, Luc, Mårtensson, Emma, Maystadt, Isabelle, Voermans, Nicol, Lederer, Damien, Burlina, Alberto, Juan Fita, María Jesús, Hoogeboom, A. Jeannette M., Olsson Engman, Mia, Hollemans, Tess, Schouten, Meyke, Meulemans, Sandra, Jonson, Tord, François, Inge, Gil Ortega, David, Kamsteeg, Erik-Jan, Creemers, John W.M.

“…PREPL deficiency causes neonatal hypotonia, ptosis, neonatal feeding difficulties, childhood obesity, xerostomia, and growth hormone deficiency. Different…”
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Loss of hypothalamic Furin affects POMC to proACTH cleavage and feeding behavior in high-fat diet-fed mice by Coppola, Ilaria, Brouwers, Bas, Walker, Leanne, Alar, Ceyhun, Meulemans, Sandra, White, Anne, Ramos-Molina, Bruno, Creemers, John W.M.

“…The hypothalamus regulates feeding and glucose homeostasis through the balanced action of different neuropeptides, which are cleaved and activated by the…”
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Knock-out mouse models of proprotein convertases: unique functions or redundancy? by Creemers, John W M, Khatib, Abdel-Majid

“…The members of the proprotein convertase family play a central role in the processing and/or activation of various protein precursors involved in many…”
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Endosome to trans-Golgi network transport of Proprotein Convertase 7 is mediated by a cluster of basic amino acids and palmitoylated cysteines by Declercq, Jeroen, Ramos-Molina, Bruno, Sannerud, Ragna, Brouwers, Bas, Pruniau, Vincent P.E.G., Meulemans, Sandra, Plets, Evelyn, Annaert, Wim, Creemers, John W.M.

“…•PC7 internalization requires a cluster of basic amino acids and two palmitoylated cysteines.•PC7 and Furin both recycle to the TGN via late endosomes.•Unlike…”
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Small-intestinal dysfunction accompanies the complex endocrinopathy of human proprotein convertase 1 deficiency by Jackson, Robert S, Creemers, John W M, Farooqi, I Sadaf, Raffin-Sanson, Marie-Laure, Varro, Andrea, Dockray, Graham J, Holst, Jens J, Brubaker, Patricia L, Corvol, Pierre, Polonsky, Kenneth S, Ostrega, Diane, Becker, Kenneth L, Bertagna, Xavier, Hutton, John C, White, Anne, Dattani, Mehul T, Hussain, Khalid, Middleton, Stephen J, Nicole, Thomasina M, Milla, Peter J, Lindley, Keith J, O'Rahilly, Stephen

“…We have previously described the only reported case of human proprotein convertase 1 (PC1) deficiency, in a female (Subject A) with obesity, hypogonadism,…”
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The proprotein convertase furin regulates the development of thymic epithelial cells to ensure central immune tolerance by Liang, Zhanfeng, Zhang, Zhaoqi, Zhang, Qian, Dong, Xue, Yang, Xiaofeng, Zhang, Jiayu, Lei, Tong, Creemers, John W.M., Zhang, Baojun, Zhao, Yong

“…The generation of mature T cells and establishment of central tolerance is predominantly orchestrated by thymic epithelial cells (TECs). Proprotein convertases…”
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SCAMP5, NBEA and AMISYN: three candidate genes for autism involved in secretion of large dense-core vesicles by Castermans, Dries, Volders, Karolien, Crepel, An, Backx, Liesbeth, De Vos, Rita, Freson, Kathleen, Meulemans, Sandra, Vermeesch, Joris R., Schrander-Stumpel, Connie T.R.M., De Rijk, Peter, Del-Favero, Jurgen, Van Geet, Chris, Van De Ven, Wim J.M., Steyaert, Jean G., Devriendt, Koen, Creemers, John W.M.

“…Autism is a neurodevelopmental disorder characterized by impaired social reciprocity, impaired communication and stereotypical behaviors. Despite strong…”
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Role of furin in granular acidification in the endocrine pancreas: Identification of the V-ATPase subunit Ac45 as a candidate substrate by Louagie, Els, Taylor, Neil A, Flamez, Daisy, Roebroek, Anton J.M, Bright, Nicholas A, Meulemans, Sandra, Quintens, Roel, Herrera, Pedro L, Schuit, Frans, Van de Ven, Wim J.M, Creemers, John W.M

“…Furin is a proprotein convertase which activates a variety of regulatory proteins in the constitutive exocytic and endocytic pathway. The effect of genetic…”
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Prolyl endopeptidase-like is a (thio)esterase involved in mitochondrial respiratory chain function by Rosier, Karen, McDevitt, Molly T., Smet, Joél, Floyd, Brendan J., Verschoore, Maxime, Marcaida, Maria J., Bingman, Craig A., Lemmens, Irma, Dal Peraro, Matteo, Tavernier, Jan, Cravatt, Benjamin F., Gounko, Natalia V., Vints, Katlijn, Monnens, Yenthe, Bhalla, Kritika, Aerts, Laetitia, Rashan, Edrees H., Vanlander, Arnaud V., Van Coster, Rudy, Régal, Luc, Pagliarini, David J., Creemers, John W.M.

“…Deficiency of the serine hydrolase prolyl endopeptidase-like (PREPL) causes a recessive metabolic disorder characterized by neonatal hypotonia, feeding…”
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A missense mutation disrupting a dibasic prohormone processing site in pro-opiomelanocortin (POMC) increases susceptibility to early-onset obesity through a novel molecular mechanism by Challis, Benjamin G., Pritchard, Lynn E., Creemers, John W.M., Delplanque, Jerome, Keogh, Julia M., Luan, Jian'an, Wareham, Nicholas J., Yeo, Giles S.H., Bhattacharyya, Sumit, Froguel, Phillipe, White, Anne, Farooqi, I. Sadaf, O'Rahilly, Stephen

“…The functional loss of both alleles of the human pro-opiomelanocortin (POMC) gene leads to a very rare syndrome of hypoadrenalism, red hair and early-onset…”
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Liver-Specific Inactivation of the Proprotein Convertase FURIN Leads to Increased Hepatocellular Carcinoma Growth by Creemers, John W. M., Khatib, Abdel-Majid, Prat, Annik, Meulemans, Sandra, Tuand, Krizia, Stijnen, Pieter, Pruniau, Vincent P. E. G., Brouwers, Bas, Declercq, Jeroen, Seidah, Nabil G.

“…Proprotein convertases are subtilisin-like serine endoproteases that cleave and hence activate a variety of proproteins, including growth factors, receptors,…”
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Obesity and impaired prohormone processing associated with mutations in the human prohormone convertase 1 gene by Creemers, John W.M, Jackson, Robert S, Montague, Carl T, Ohagi, Shinya, Raffin-Sanson, Marie-Laure, Hutton, John C, O'Rahilly, Stephen, Sanders, Louise

“…Human obesity has an inherited component, but in contrast to rodent obesity, precise genetic defects have yet to be defined. A mutation of carboxypeptidase E…”
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Limited Redundancy of the Proprotein Convertase Furin in Mouse Liver by Roebroek, Anton J.M., Taylor, Neil A., Louagie, Els, Pauli, Ilse, Smeijers, Liesbeth, Snellinx, An, Lauwers, Annick, Van de Ven, Wim J.M., Hartmann, Dieter, Creemers, John W.M.

“…Furin is an endoprotease of the family of mammalian proprotein convertases and is involved in the activation of a large variety of regulatory proteins by…”
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