{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T12:11:39Z","timestamp":1787314299668,"version":"build-2736575974"},"reference-count":53,"publisher":"Public Library of Science (PLoS)","issue":"3","license":[{"start":{"date-parts":[[2015,3,11]],"date-time":"2015-03-11T00:00:00Z","timestamp":1426032000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0119455","type":"journal-article","created":{"date-parts":[[2015,3,11]],"date-time":"2015-03-11T13:47:26Z","timestamp":1426081646000},"page":"e0119455","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":14,"title":["T396I Mutation of Mouse Sufu Reduces the Stability and Activity of Gli3 Repressor"],"prefix":"10.1371","volume":"10","author":[{"given":"Shigeru","family":"Makino","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olena","family":"Zhulyn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rong","family":"Mo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vijitha","family":"Puviindran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyun","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuya","family":"Murata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryutaro","family":"Fukumura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuichi","family":"Ishitsuka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hayato","family":"Kotaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daisuke","family":"Matsumaru","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunsuke","family":"Ishii","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chi-Chung","family":"Hui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoichi","family":"Gondo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2015,3,11]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1016\/j.devcel.2008.11.010","article-title":"Hedgehog signaling in development and cancer","volume":"15","author":"J Jiang","year":"2008","journal-title":"Dev Cell"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1038\/nrm3598","article-title":"The mechanisms of Hedgehog signalling and its roles in development and disease","volume":"14","author":"J Briscoe","year":"2013","journal-title":"Nat Rev Mol Cell Biol"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"a002014","DOI":"10.1101\/cshperspect.a002014","article-title":"Establishing and interpreting graded Sonic Hedgehog signaling during vertebrate neural tube patterning: the role of negative feedback","volume":"1","author":"V Ribes","year":"2009","journal-title":"Cold Spring Harb Perspect Biol"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1016\/j.gde.2010.04.014","article-title":"The temporal dynamics of vertebrate limb development, teratogenesis and evolution","volume":"20","author":"R Zeller","year":"2010","journal-title":"Curr Opin Genet Dev"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1146\/annurev-cellbio-092910-154048","article-title":"Gli proteins in development and disease","volume":"27","author":"CC Hui","year":"2011","journal-title":"Annu Rev Cell Dev Biol"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1242\/dev.124.1.113","article-title":"Specific and redundant functions of Gli2 and Gli3 zinc finger genes in skeletal patterning and development","volume":"124","author":"R Mo","year":"1997","journal-title":"Development"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"2533","DOI":"10.1242\/dev.125.14.2533","article-title":"Diminished Sonic hedgehog signaling and lack of floor plate differentiation in Gli2 mutant mice","volume":"125","author":"Q Ding","year":"1998","journal-title":"Development"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"1593","DOI":"10.1242\/dev.127.8.1593","article-title":"Mouse Gli1 mutants are viable but have defects in SHH signaling in combination with a Gli2 mutation","volume":"127","author":"HL Park","year":"2000","journal-title":"Development"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"4753","DOI":"10.1242\/dev.129.20.4753","article-title":"Gli2, but not Gli1, is required for initial Shh signaling and ectopic activation of the Shh pathway","volume":"129","author":"CB Bai","year":"2002","journal-title":"Development"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1038\/ng0393-241","article-title":"A mouse model of greig cephalopolysyndactyly syndrome: the extra-toesJ mutation contains an intragenic deletion of the Gli3 gene","volume":"3","author":"CC Hui","year":"1993","journal-title":"Nat Genet"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"2865","DOI":"10.1101\/gad.243402","article-title":"Dorsal-ventral patterning of the spinal cord requires Gli3 transcriptional repressor activity","volume":"16","author":"M Persson","year":"2002","journal-title":"Genes Dev"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"8143","DOI":"10.1074\/jbc.274.12.8143","article-title":"Sonic Hedgehog-induced activation of the Gli1 promoter is mediated by GLI3","volume":"274","author":"P Dai","year":"1999","journal-title":"J Biol Chem"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"3915","DOI":"10.1242\/dev.126.17.3915","article-title":"Regulation of Gli2 and Gli3 activities by an amino-terminal repression domain: implication of Gli2 and Gli3 as primary mediators of Shh signaling","volume":"126","author":"H Sasaki","year":"1999","journal-title":"Development"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"3365","DOI":"10.1128\/MCB.26.9.3365-3377.2006","article-title":"Sonic hedgehog signaling regulates Gli2 transcriptional activity by suppressing its processing and degradation","volume":"26","author":"Y Pan","year":"2006","journal-title":"Mol Cell Biol"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"670","DOI":"10.1101\/gad.1902910","article-title":"The output of Hedgehog signaling is controlled by the dynamic association between Suppressor of Fused and the Gli proteins","volume":"24","author":"EW Humke","year":"2010","journal-title":"Genes Dev"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"1910","DOI":"10.1101\/gad.1794109","article-title":"Cilium-independent regulation of Gli protein function by Sufu in Hedgehog signaling is evolutionarily conserved","volume":"23","author":"MH Chen","year":"2009","journal-title":"Genes Dev"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"1910","DOI":"10.1128\/MCB.01089-09","article-title":"Kinetics of hedgehog-dependent full-length Gli3 accumulation in primary cilia and subsequent degradation","volume":"30","author":"X Wen","year":"2010","journal-title":"Mol Cell Biol"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"4316","DOI":"10.1128\/MCB.02183-05","article-title":"Multisite protein kinase A and glycogen synthase kinase 3beta phosphorylation leads to Gli3 ubiquitination by SCFbetaTrCP","volume":"26","author":"D Tempe","year":"2006","journal-title":"Mol Cell Biol"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1073\/pnas.0509927103","article-title":"Evidence for the direct involvement of {beta}TrCP in Gli3 protein processing","volume":"103","author":"B Wang","year":"2006","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"2001","DOI":"10.1242\/dev.052126","article-title":"Suppressor of fused and Spop regulate the stability, processing and function of Gli2 and Gli3 full-length activators but not their repressors","volume":"137","author":"C Wang","year":"2010","journal-title":"Development"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/j.bbrc.2009.07.087","article-title":"Sufu recruits GSK3beta for efficient processing of Gli3","volume":"387","author":"Y Kise","year":"2009","journal-title":"Biochem Biophys Res Commun"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"4407","DOI":"10.1242\/dev.02021","article-title":"Cardiac and CNS defects in a mouse with targeted disruption of suppressor of fused","volume":"132","author":"AF Cooper","year":"2005","journal-title":"Development"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1016\/j.devcel.2005.12.013","article-title":"Genetic elimination of suppressor of fused reveals an essential repressor function in the Mammalian hedgehog signaling pathway","volume":"10","author":"J Svard","year":"2006","journal-title":"Dev Cell"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/j.ydbio.2011.11.022","article-title":"Dual function of suppressor of fused in Hh pathway activation and mouse spinal cord patterning","volume":"362","author":"J Liu","year":"2012","journal-title":"Dev Biol"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1038\/nrg2431","article-title":"Trends in large-scale mouse mutagenesis: from genetics to functional genomics","volume":"9","author":"Y Gondo","year":"2008","journal-title":"Nat Rev Genet"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"8627","DOI":"10.1128\/MCB.24.19.8627-8641.2004","article-title":"Suppressor of fused regulates Gli activity through a dual binding mechanism","volume":"24","author":"M Merchant","year":"2004","journal-title":"Mol Cell Biol"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1006\/dbio.1997.8644","article-title":"Evidence for the involvement of the Gli gene family in embryonic mouse lung development","volume":"188","author":"JC Grindley","year":"1997","journal-title":"Dev Biol"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1016\/j.ydbio.2009.02.017","article-title":"A SHH-independent regulation of Gli3 is a significant determinant of anteroposterior patterning of the limb bud","volume":"328","author":"P Hill","year":"2009","journal-title":"Dev Biol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"1218","DOI":"10.1016\/j.mod.2005.06.012","article-title":"Pax9 and Jagged1 act downstream of Gli3 in vertebrate limb development","volume":"122","author":"E McGlinn","year":"2005","journal-title":"Mech Dev"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/S0925-4773(97)00656-4","article-title":"Evidence for genetic control of Sonic hedgehog by Gli3 in mouse limb development","volume":"62","author":"D Buscher","year":"1997","journal-title":"Mech Dev"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1101\/gad.219202","article-title":"Mutual genetic antagonism involving GLI3 and dHAND prepatterns the vertebrate limb bud mesenchyme prior to SHH signaling","volume":"16","author":"P te Welscher","year":"2002","journal-title":"Genes Dev"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"e1000901","DOI":"10.1371\/journal.pgen.1000901","article-title":"Distinct roles of Hand2 in initiating polarity and posterior Shh expression during the onset of mouse limb bud development","volume":"6","author":"A Galli","year":"2010","journal-title":"PLoS Genet"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/j.devcel.2014.03.002","article-title":"A switch from low to high Shh activity regulates establishment of limb progenitors and signaling centers","volume":"29","author":"O Zhulyn","year":"2014","journal-title":"Dev Cell"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1093\/hmg\/11.9.1129","article-title":"Pallister-Hall syndrome phenotype in mice mutant for Gli3","volume":"11","author":"J Bose","year":"2002","journal-title":"Hum Mol Genet"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"5161","DOI":"10.1242\/dev.128.24.5161","article-title":"Gli1 can rescue the in vivo function of Gli2","volume":"128","author":"CB Bai","year":"2001","journal-title":"Development"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1038\/79916","article-title":"Specification of ventral neuron types is mediated by an antagonistic interaction between Shh and Gli3","volume":"3","author":"Y Litingtung","year":"2000","journal-title":"Nat Neurosci"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1016\/S0092-8674(01)00385-3","article-title":"Smoothened mutants reveal redundant roles for Shh and Ihh signaling including regulation of L\/R symmetry by the mouse node","volume":"106","author":"XM Zhang","year":"2001","journal-title":"Cell"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"1628","DOI":"10.1016\/S0960-9822(02)01147-8","article-title":"Mouse Dispatched homolog1 is required for long-range, but not juxtacrine, Hh signaling","volume":"12","author":"T Caspary","year":"2002","journal-title":"Curr Biol"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"2849","DOI":"10.1101\/gad.1025702","article-title":"A direct requirement for Hedgehog signaling for normal specification of all ventral progenitor domains in the presumptive mammalian spinal cord","volume":"16","author":"M Wijgerde","year":"2002","journal-title":"Genes Dev"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1016\/j.ydbio.2009.04.009","article-title":"Suppressor of Fused inhibits mammalian Hedgehog signaling in the absence of cilia","volume":"330","author":"J Jia","year":"2009","journal-title":"Dev Biol"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"e50193","DOI":"10.1371\/journal.pone.0050193","article-title":"Antagonistic and cooperative actions of Kif7 and Sufu define graded intracellular Gli activities in Hedgehog signaling","volume":"7","author":"KK Law","year":"2012","journal-title":"PLoS One"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1016\/S0960-9822(99)80482-5","article-title":"Mouse suppressor of fused is a negative regulator of sonic hedgehog signaling and alters the subcellular distribution of Gli1","volume":"9","author":"Q Ding","year":"1999","journal-title":"Curr Biol"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1038\/13031","article-title":"Mammalian suppressor-of-fused modulates nuclear-cytoplasmic shuttling of Gli-1","volume":"1","author":"P Kogerman","year":"1999","journal-title":"Nat Cell Biol"},{"issue":"Pt 23","key":"ref44","doi-asserted-by":"crossref","first-page":"4437","DOI":"10.1242\/jcs.112.23.4437","article-title":"Characterization of the human suppressor of fused, a negative regulator of the zinc-finger transcription factor Gli","volume":"112","author":"DM Stone","year":"1999","journal-title":"J Cell Sci"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1038\/35010610","article-title":"Gli regulation by the opposing activities of fused and suppressor of fused","volume":"2","author":"M Murone","year":"2000","journal-title":"Nat Cell Biol"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1111\/j.1432-0436.2005.00042.x","article-title":"Negative regulation of Gli1 and Gli2 activator function by Suppressor of fused through multiple mechanisms","volume":"73","author":"PC Barnfield","year":"2005","journal-title":"Differentiation"},{"key":"ref47","doi-asserted-by":"crossref","first-page":"10846","DOI":"10.1074\/jbc.M608599200","article-title":"A novel protein-processing domain in Gli2 and Gli3 differentially blocks complete protein degradation by the proteasome","volume":"282","author":"Y Pan","year":"2007","journal-title":"J Biol Chem"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"2608","DOI":"10.1038\/ncomms3608","article-title":"Structural insight into the mutual recognition and regulation between Suppressor of Fused and Gli\/Ci","volume":"4","author":"Y Zhang","year":"2013","journal-title":"Nat Commun"},{"key":"ref49","doi-asserted-by":"crossref","first-page":"2563","DOI":"10.1107\/S0907444913028473","article-title":"Structural basis of SUFU-GLI interaction in human Hedgehog signalling regulation","volume":"69","author":"AL Cherry","year":"2013","journal-title":"Acta Crystallogr D Biol Crystallogr"},{"key":"ref50","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1016\/0092-8674(90)90492-W","article-title":"What determines the specificity of action of Drosophila homeodomain proteins?","volume":"63","author":"S Hayashi","year":"1990","journal-title":"Cell"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/j.cell.2009.12.027","article-title":"Drosophila genome-wide obesity screen reveals hedgehog as a determinant of brown versus white adipose cell fate","volume":"140","author":"JA Pospisilik","year":"2010","journal-title":"Cell"},{"key":"ref52","doi-asserted-by":"crossref","first-page":"40113","DOI":"10.1074\/jbc.M105317200","article-title":"Suppressor of fused negatively regulates beta-catenin signaling","volume":"276","author":"X Meng","year":"2001","journal-title":"J Biol Chem"},{"key":"ref53","doi-asserted-by":"crossref","first-page":"ra29","DOI":"10.1126\/scisignal.2000405","article-title":"The kinesin protein Kif7 is a critical regulator of Gli transcription factors in mammalian hedgehog signaling","volume":"2","author":"HO Cheung","year":"2009","journal-title":"Sci Signal"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0119455","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,5,1]],"date-time":"2022-05-01T20:28:46Z","timestamp":1651436926000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0119455"}},"subtitle":[],"editor":[{"given":"Martin","family":"Fernandez-Zapico","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2015,3,11]]},"references-count":53,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2015,3,11]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0119455","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,3,11]]}}}