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https://www.readbyqxmd.com/read/26933123/gemc1-is-a-critical-regulator-of-multiciliated-cell-differentiation
#1
Berta Terré, Gabriele Piergiovanni, Sandra Segura-Bayona, Gabriel Gil-Gómez, Sameh A Youssef, Camille Stephan-Otto Attolini, Michaela Wilsch-Bräuninger, Carole Jung, Ana M Rojas, Marko Marjanović, Philip A Knobel, Lluís Palenzuela, Teresa López-Rovira, Stephen Forrow, Wieland B Huttner, Miguel A Valverde, Alain de Bruin, Vincenzo Costanzo, Travis H Stracker
The generation of multiciliated cells (MCCs) is required for the proper function of many tissues, including the respiratory tract, brain, and germline. Defects in MCC development have been demonstrated to cause a subclass of mucociliary clearance disorders termed reduced generation of multiple motile cilia (RGMC). To date, only two genes, Multicilin (MCIDAS) and cyclin O (CCNO) have been identified in this disorder in humans. Here, we describe mice lacking GEMC1 (GMNC), a protein with a similar domain organization as Multicilin that has been implicated in DNA replication control...
May 2, 2016: EMBO Journal
https://www.readbyqxmd.com/read/26839325/quantification-of-hepcidin-related-iron-accumulation-in-the-rat-liver
#2
Preethne Böser, Yulia Mordashova, Mark Maasland, Isabel Trommer, Helga Lorenz, Mathias Hafner, Dietmar Seemann, Bernhard K Mueller, Andreas Popp
Hepcidin was originally detected as a liver peptide with antimicrobial activity and it functions as a central regulator in the systemic iron metabolism. Consequently suppression of hepcidin leads to iron accumulation in the liver. AbbVie developed a monoclonal antibody ([mAb]; repulsive guidance molecule [RGMa/c] mAb) that downregulates hepcidin expression by influencing the RGMc/bone morphogenetic protein (BMP)/neogenin receptor complex and causes iron deposition in the liver. In a dose range finding study with RGMa/c mAb, rats were treated with different dose levels for a total of 4 weekly doses...
February 2016: Toxicologic Pathology
https://www.readbyqxmd.com/read/26777464/systematic-analysis-of-ccno-variants-in-a-defined-population-implications-for-clinical-phenotype-and-differential-diagnosis
#3
Israel Amirav, Julia Wallmeier, Niki T Loges, Tabea Menchen, Petra Pennekamp, Huda Mussaffi, Revital Abitbul, Avraham Avital, Lea Bentur, Gerard W Dougherty, Elias Nael, Moran Lavie, Heike Olbrich, Claudius Werner, Chris Kintner, Heymut Omran
Reduced generation of multiple motile cilia (RGMC) is a novel chronic destructive airway disease within the group of mucociliary clearance disorders with only few cases reported. Mutations in two genes, CCNO and MCIDAS, have been identified as a cause of this disease, both leading to a greatly reduced number of cilia and causing impaired mucociliary clearance. This study was designed to identify the prevalence of CCNO mutations in Israel and further delineate the clinical characteristics of RGMC. We analyzed 170 families with mucociliary clearance disorders originating from Israel for mutations in CCNO and identified two novel mutations (c...
April 2016: Human Mutation
https://www.readbyqxmd.com/read/25704252/identification-of-tmprss6-cleavage-sites-of-hemojuvelin
#4
Marco Rausa, Michela Ghitti, Alessia Pagani, Antonella Nai, Alessandro Campanella, Giovanna Musco, Clara Camaschella, Laura Silvestri
Hemojuvelin (HJV), the coreceptor of the BMP-SMAD pathway that up-regulates hepcidin transcription, is a repulsive guidance molecule (RGMc) which undergoes a complex intracellular processing. Following autoproteolysis, it is exported to the cell surface both as a full-length and a heterodimeric protein. In vitro membrane HJV (m-HJV) is cleaved by the transmembrane serine protease TMPRSS6 to attenuate signalling and to inhibit hepcidin expression. In this study, we investigated the number and position of HJV cleavage sites by mutagenizing arginine residues (R), potential TMPRSS6 targets, to alanine (A)...
April 2015: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/25048963/mcidas-mutations-result-in-a-mucociliary-clearance-disorder-with-reduced-generation-of-multiple-motile-cilia
#5
Mieke Boon, Julia Wallmeier, Lina Ma, Niki Tomas Loges, Martine Jaspers, Heike Olbrich, Gerard W Dougherty, Johanna Raidt, Claudius Werner, Israel Amirav, Avigdor Hevroni, Revital Abitbul, Avraham Avital, Ruth Soferman, Marja Wessels, Christopher O'Callaghan, Eddie M K Chung, Andrew Rutman, Robert A Hirst, Eduardo Moya, Hannah M Mitchison, Sabine Van Daele, Kris De Boeck, Mark Jorissen, Chris Kintner, Harry Cuppens, Heymut Omran
Reduced generation of multiple motile cilia (RGMC) is a rare mucociliary clearance disorder. Affected persons suffer from recurrent infections of upper and lower airways because of highly reduced numbers of multiple motile respiratory cilia. Here we report recessive loss-of-function and missense mutations in MCIDAS-encoding Multicilin, which was shown to promote the early steps of multiciliated cell differentiation in Xenopus. MCIDAS mutant respiratory epithelial cells carry only one or two cilia per cell, which lack ciliary motility-related proteins (DNAH5; CCDC39) as seen in primary ciliary dyskinesia...
2014: Nature Communications
https://www.readbyqxmd.com/read/25013897/neogenin-as-a-receptor-for-early-cell-fate-determination-in-preimplantation-mouse-embryos
#6
Jae Ho Lee, Sung Sook Choi, Hae-Won Kim, Hae Won Kim, Wen Cheng Xiong, Churl K Min, Sang Jin Lee
The first cell lineage determination in embryos takes place when two cell populations are set apart, each differentiating into the trophectoderm (TE) and inner cell mass (ICM), respectively. It is widely believed that position/polarity cues play a key role in triggering this differentiation, but it remains unclear how extracellular cues are transduced into cell fate determination. Here, we provide evidence that supports that neogenin is implicated in relaying extracellular cues into the first cell fate determination in preimplantation mouse embryos...
2014: PloS One
https://www.readbyqxmd.com/read/24052264/dragon-repulsive-guidance-molecule-rgmb-inhibits-e-cadherin-expression-and-induces-apoptosis-in-renal-tubular-epithelial-cells
#7
Wenjing Liu, Xiaoling Li, Yueshui Zhao, Xiao-Ming Meng, Chao Wan, Baoxue Yang, Hui-Yao Lan, Herbert Y Lin, Yin Xia
Dragon is one of the three members of the repulsive guidance molecule (RGM) family, i.e. RGMa, RGMb (Dragon), and RGMc (hemojuvelin). We previously identified the RGM members as bone morphogenetic protein (BMP) co-receptors that enhance BMP signaling. Our previous studies found that Dragon is highly expressed in the tubular epithelial cells of mouse kidneys. However, the roles of Dragon in renal epithelial cells are yet to be defined. We now show that overexpression of Dragon increased cell death induced by hypoxia in association with increased cleaved poly(ADP-ribose) polymerase and cleaved caspase-3 levels in mouse inner medullary collecting duct (IMCD3) cells...
November 1, 2013: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/23464809/proteomic-analysis-and-molecular-modelling-characterize-the-iron-regulatory-protein-haemojuvelin-repulsive-guidance-molecule-c
#8
Mahta Nili, Larry David, Johannes Elferich, Ujwal Shinde, Peter Rotwein
HJV (haemojuvelin) plays a key role in iron metabolism in mammals by regulating expression of the liver-derived hormone hepcidin, which controls systemic iron uptake and release. Mutations in HJV cause juvenile haemochromatosis, a rapidly progressing iron overload disorder in humans. HJV, also known as RGMc (repulsive guidance molecule c), is a member of the three-protein RGM family. RGMs are GPI (glycosylphosphatidylinositol)-linked glycoproteins that share ~50% amino acid identity and several structural motifs, including the presence of 14 cysteine residues in analogous locations...
May 15, 2013: Biochemical Journal
https://www.readbyqxmd.com/read/23073896/rgma-and-rgmb-expression-pattern-during-chicken-development-suggest-unexpected-roles-for-these-repulsive-guidance-molecules-in-notochord-formation-somitogenesis-and-myogenesis
#9
Erika Cristina Jorge, Mohi U Ahmed, Ingo Bothe, Luiz Lehmman Coutinho, Susanne Dietrich
BACKGROUND: Repulsive guidance molecules (RGM) are high-affinity ligands for the Netrin receptor Neogenin, and they are crucial for nervous system development including neural tube closure; neuronal and neural crest cell differentiation and axon guidance. Recent studies implicated RGM molecules in bone morphogenetic protein signaling, which regulates a variety of developmental processes. Moreover, a role for RGMc in iron metabolism has been established. This suggests that RGM molecules may play important roles in non-neural tissues...
December 2012: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/23029472/repulsive-guidance-molecule-rgm-family-proteins-exhibit-differential-binding-kinetics-for-bone-morphogenetic-proteins-bmps
#10
Qifang Wu, Chia Chi Sun, Herbert Y Lin, Jodie L Babitt
Bone morphogenetic proteins (BMPs) are members of the transforming growth factor beta superfamily that exert their effects via type I and type II serine threonine kinase receptors and the SMAD intracellular signaling pathway to regulate diverse biologic processes. Recently, we discovered that the repulsive guidance molecule (RGM) family, including RGMA, RGMB, and RGMC/hemojuvelin (HJV), function as co-receptors that enhance cellular responses to BMP ligands. Here, we use surface plasmon resonance to quantitate the binding kinetics of RGM proteins for BMP ligands...
2012: PloS One
https://www.readbyqxmd.com/read/22403779/distribution-of-cortical-benzodiazepine-receptor-binding-in-right-handed-healthy-humans-a-voxel-based-statistical-analysis-of-iodine-123-iomazenil-spect-with-partial-volume-correction
#11
H Kato, E Shimosegawa, K Isohashi, N Kimura, H Kazui, J Hatazawa
BACKGROUND AND PURPOSE: CBR imaging is highly susceptible to a PVE produced by morphologic changes in the brain related to aging and brain laterality. We assessed the influence of PVE produced by regional age-related changes in gray matter volume on I-123 iomazenil SPECT and elucidated the age-related changes in human CBR binding by using PVE-corrected SPECT images. MATERIALS AND METHODS: Nineteen right-handed healthy volunteers (range, 25-82 years; mean, 55 ± 21 years) underwent MR imaging and quantitative I-123 iomazenil SPECT imaging...
September 2012: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/22076499/repulsive-guidance-molecules-novel-bone-morphogenetic-protein-co-receptors-are-key-regulators-of-the-growth-and-aggressiveness-of-prostate-cancer-cells
#12
Jin Li, Lin Ye, Howard G Kynaston, Wen G Jiang
Repulsive guidance molecule (RGM) family members RGMA, RGMB and RGMC are GPI-linked membrane proteins recently identified as co-receptor of bone morphogenetic proteins (BMPs). BMPs are a group of proteins enriched in bone and play important roles in prostate cancer. The current study aimed to investigate roles played by RGMs in prostate cancer. Expression of RGMs was examined in prostate cancer cell lines and prostate cancer tissues using RT-PCR and immunohistochemical staining. Knockdown of each RGM in prostate cancer cells was performed using the respective anti-RGMA, RGMB and RGMC transgenes...
February 2012: International Journal of Oncology
https://www.readbyqxmd.com/read/21617229/potential-prognostic-value-of-repulsive-guidance-molecules-in-breast-cancer
#13
Jin Li, Lin Ye, Robert E Mansel, Wen G Jiang
BACKGROUND: Repulsive guidance molecules (RGMs) are novel co-receptors of bone morphogenetic proteins (BMPs) which have been implicated in bone metastasis of cancer. This study aimed to investigate roles played by RGMs in breast cancer. MATERIALS AND METHODS: Expression of RGMs was examined in breast cancer cell lines using RT-PCR. The expression of RGMs in human breast cancer tissues was assessed using both quantitative PCR and immunohistochemical staining. RESULTS: RGMB was detectable in both cell lines and tissues samples of breast cancer...
May 2011: Anticancer Research
https://www.readbyqxmd.com/read/21187450/dragon-repulsive-guidance-molecule-b-inhibits-il-6-expression-in-macrophages
#14
Yin Xia, Virna Cortez-Retamozo, Vera Niederkofler, Rishard Salie, Shanzhuo Chen, Tarek A Samad, Charles C Hong, Silvia Arber, Jatin M Vyas, Ralph Weissleder, Mikael J Pittet, Herbert Y Lin
Repulsive guidance molecule (RGM) family members RGMa, RGMb/Dragon, and RGMc/hemojuvelin were found recently to act as bone morphogenetic protein (BMP) coreceptors that enhance BMP signaling activity. Although our previous studies have shown that hemojuvelin regulates hepcidin expression and iron metabolism through the BMP pathway, the role of the BMP signaling mediated by Dragon remains largely unknown. We have shown previously that Dragon is expressed in neural cells, germ cells, and renal epithelial cells...
February 1, 2011: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/21113736/development-of-a-transgenic-zebrafish-model-expressing-gfp-in-the-notochord-somite-and-liver-directed-by-the-hfe2-gene-promoter
#15
Yue-Hong Bian, Cheng Xu, Junling Li, Jin Xu, Hongwei Zhang, Shao Jun Du
Hemojuvelin, also known as RGMc, is encoded by hfe2 gene that plays an important role in iron homeostasis. hfe2 is specifically expressed in the notochord, developing somite and skeletal muscles during development. The molecular regulation of hfe2 expression is, however, not clear. We reported here the characterization of hfe2 gene expression and the regulation of its tissue-specific expression in zebrafish embryos. We demonstrated that the 6 kb 5'-flanking sequence upstream of the ATG start codon in the zebrafish hfe2 gene could direct GFP specific expression in the notochord, somites, and skeletal muscle of zebrafish embryos, recapitulating the expression pattern of the endogenous gene...
August 2011: Transgenic Research
https://www.readbyqxmd.com/read/20858542/conserved-proximal-promoter-elements-control-repulsive-guidance-molecule-c-hemojuvelin-hfe2-gene-transcription-in-skeletal-muscle
#16
Christopher J Severyn, Peter Rotwein
Repulsive guidance molecule c (RGMc; gene symbol: Hfe2) plays a critical role in iron metabolism. Inactivating mutations cause juvenile hemochromatosis, a severe iron overload disorder. Understanding mechanisms controlling RGMc biosynthesis has been hampered by minimal information about the RGMc gene. Here we define the structure, examine the evolution, and establish mechanisms of regulation of the mouse RGMc gene. RGMc is a 4-exon gene that undergoes alternative RNA splicing to yield 3 mRNAs with 5' different untranslated regions...
December 2010: Genomics
https://www.readbyqxmd.com/read/20530805/soluble-repulsive-guidance-molecule-c-hemojuvelin-is-a-broad-spectrum-bone-morphogenetic-protein-bmp-antagonist-and-inhibits-both-bmp2-and-bmp6-mediated-signaling-and-gene-expression
#17
Mahta Nili, Ujwal Shinde, Peter Rotwein
Inactivating mutations in hemojuvelin/repulsive guidance molecule c (HJV/RGMc) cause juvenile hemochromatosis (JH), a rapidly progressive iron overload disorder in which expression of hepcidin, a key liver-derived iron-regulatory hormone, is severely diminished. Several growth factors in the bone morphogenetic protein (BMP) family, including BMP2 and BMP6, can stimulate production of hepcidin, a biological effect that may be modified by RGMc. Here we demonstrate that soluble RGMc proteins are potent BMP inhibitors...
August 6, 2010: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/20065295/neogenin-inhibits-hjv-secretion-and-regulates-bmp-induced-hepcidin-expression-and-iron-homeostasis
#18
Dae-Hoon Lee, Dai-Hoon Lee, Li-Juan Zhou, Li-Juau Zhou, Zheng Zhou, Jian-Xin Xie, Jiau-Xiu Xie, Ji-Ung Jung, Yu Liu, Cai-Xia Xi, Lin Mei, Wen-Cheng Xiong
Neogenin, a deleted in colorectal cancer (DCC) family member, has been identified as a receptor for the neuronal axon guidance cues netrins and repulsive guidance molecules repulsive guidance molecules (RGM). RGMc, also called hemojuvelin (HJV), is essential for iron homeostasis. Here we provide evidence that neogenin plays a critical role in iron homeostasis by regulation of HJV secretion and bone morphogenetic protein (BMP) signaling. Livers of neogenin mutant mice exhibit iron overload, low levels of hepcidin, and reduced BMP signaling...
April 15, 2010: Blood
https://www.readbyqxmd.com/read/19812254/neogenin-regulates-skeletal-myofiber-size-and-focal-adhesion-kinase-and-extracellular-signal-regulated-kinase-activities-in-vivo-and-in-vitro
#19
Gyu-Un Bae, Youn-Joo Yang, Guoying Jiang, Mingi Hong, Hye-Jin Lee, Marc Tessier-Lavigne, Jong-Sun Kang, Robert S Krauss
A variety of signaling pathways participate in the development of skeletal muscle, but the extracellular cues that regulate such pathways in myofiber formation are not well understood. Neogenin is a receptor for ligands of the netrin and repulsive guidance molecule (RGM) families involved in axon guidance. We reported previously that neogenin promoted myotube formation by C2C12 myoblasts in vitro and that the related protein Cdo (also Cdon) was a potential neogenin coreceptor in myoblasts. We report here that mice homozygous for a gene-trap mutation in the Neo1 locus (encoding neogenin) develop myotomes normally but have small myofibers at embryonic day 18...
December 2009: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/19698085/molecular-biology-genetics-and-biochemistry-of-the-repulsive-guidance-molecule-family
#20
REVIEW
Christopher J Severyn, Ujwal Shinde, Peter Rotwein
RGMs (repulsive guidance molecules) comprise a recently discovered family of GPI (glycosylphosphatidylinositol)-linked cell-membrane-associated proteins found in most vertebrate species. The three proteins, RGMa, RGMb and RGMc, products of distinct single-copy genes that arose early in vertebrate evolution, are approximately 40-50% identical to each other in primary amino acid sequence, and share similarities in predicted protein domains and overall structure, as inferred by ab initio molecular modelling; yet the respective proteins appear to undergo distinct biosynthetic and processing steps, whose regulation has not been characterized to date...
August 27, 2009: Biochemical Journal
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