Gene Symbol: Xbp1
Description: X box binding protein-1
Alias: 138/3, CG 9415, CG9415, DM18, Dmel\CG9415, Dxbp-1, XBP-1, XBP1, Xbp-1, XbpI, anon-EST:Liang-1.41, clone 1.41, dXbp-1, l(2)k13803, xbp-1, xbp1, X box binding protein-1, CG9415-PD, CG9415-PE, X box binding protein 1, X-box binding protein, X-box-binding protein 1, Xbp1-PD, Xbp1-PE
Species: fruit fly
Products:     Xbp1

Top Publications

  1. Souid S, Lepesant J, Yanicostas C. The xbp-1 gene is essential for development in Drosophila. Dev Genes Evol. 2007;217:159-67 pubmed
    ..The Dxbp-1 gene appears, thus, to play an essential role during the development of Drosophila, hypothetically by stimulating the folding capacities of the ER in cells committed to intense secretory activities. ..
  2. Moore K, Plant J, Gaddam D, Craft J, Hollien J. Regulation of sumo mRNA during endoplasmic reticulum stress. PLoS ONE. 2013;8:e75723 pubmed publisher
    ..Ire1's nuclease initiates the cytosolic splicing of the mRNA encoding X-box binding protein (Xbp1), a potent transcription factor that then upregulates genes responsible for restoring ER function...
  3. Kang M, Ryoo H. Suppression of retinal degeneration in Drosophila by stimulation of ER-associated degradation. Proc Natl Acad Sci U S A. 2009;106:17043-8 pubmed publisher
    ..These results suggest that manipulation of ERAD may serve as a powerful therapeutic strategy against a number of diseases associated with ER stress. ..
  4. Chow C, Wolfner M, Clark A. Using natural variation in Drosophila to discover previously unknown endoplasmic reticulum stress genes. Proc Natl Acad Sci U S A. 2013;110:9013-8 pubmed publisher
    ..Survival time was significantly associated with polymorphisms in candidate genes with known (i.e., Xbp1) and unknown roles in ER stress...
  5. Suzuki T, Shimoda M, Ito K, Hanai S, Aizawa H, Kato T, et al. Expression of human Gaucher disease gene GBA generates neurodevelopmental defects and ER stress in Drosophila eye. PLoS ONE. 2013;8:e69147 pubmed publisher
    ..We demonstrate a novel mechanism of neurodevelopmental defects mediated by ER stress through expression of mutants of human GBA gene in the eye of Drosophila. ..
  6. Ryoo H, Domingos P, Kang M, Steller H. Unfolded protein response in a Drosophila model for retinal degeneration. EMBO J. 2007;26:242-52 pubmed
    ..Here, we analyzed a branch of the UPR mediated by xbp1 in Drosophila to establish its role in neurodegenerative diseases...
  7. Kumita J, Helmfors L, Williams J, Luheshi L, Menzer L, Dumoulin M, et al. Disease-related amyloidogenic variants of human lysozyme trigger the unfolded protein response and disturb eye development in Drosophila melanogaster. FASEB J. 2012;26:192-202 pubmed publisher
  8. Casas Tinto S, Zhang Y, Sanchez Garcia J, Gomez Velazquez M, Rincon Limas D, Fernandez Funez P. The ER stress factor XBP1s prevents amyloid-beta neurotoxicity. Hum Mol Genet. 2011;20:2144-60 pubmed publisher
    ..We report here that A? activates the ER stress response factor X-box binding protein 1 (XBP1) in transgenic flies and in mammalian cultured neurons, yielding its active form, the transcription factor XBP1s...
  9. Mendes C, Levet C, Chatelain G, Dourlen P, Fouillet A, Dichtel Danjoy M, et al. ER stress protects from retinal degeneration. EMBO J. 2009;28:1296-307 pubmed publisher
    ..We propose that an immediate consequence of the UPR not only limits the accumulation of misfolded proteins but also protects tissues from harmful exogenous stresses. ..

More Information


  1. Sone M, Zeng X, Larese J, Ryoo H. A modified UPR stress sensing system reveals a novel tissue distribution of IRE1/XBP1 activity during normal Drosophila development. Cell Stress Chaperones. 2013;18:307-19 pubmed publisher
    ..In Drosophila, IRE1/XBP1 pathway is considered to function as a major branch of UPR; however, its physiological roles during the normal ..
  2. Garrey J, Lee Y, Au H, Bushell M, Jan E. Host and viral translational mechanisms during cricket paralysis virus infection. J Virol. 2010;84:1124-38 pubmed publisher
    ..These results support a model by which the alteration of the deIF4F complex contribute to the shutoff of host translation during CrPV infection, thereby promoting viral protein synthesis via the CrPV 5'UTR and IGR IRES. ..
  3. Ryoo H, Steller H. Unfolded protein response in Drosophila: why another model can make it fly. Cell Cycle. 2007;6:830-5 pubmed
    ..Here, we review the molecular components of the Drosophila UPR as well as the disease models that may be affected by this signaling pathway. ..
  4. Brown M, Chan M, Zimmerman J, Pack A, Jackson N, Naidoo N. Aging induced endoplasmic reticulum stress alters sleep and sleep homeostasis. Neurobiol Aging. 2014;35:1431-41 pubmed publisher
    ..Alleviating prolonged or sustained ER stress during aging contributes to sleep consolidation and improves recovery sleep or sleep debt discharge. ..
  5. Ryoo H, Li J, Kang M. Drosophila XBP1 expression reporter marks cells under endoplasmic reticulum stress and with high protein secretory load. PLoS ONE. 2013;8:e75774 pubmed publisher
    ..Upon ER stress-induced activation, IRE1 splices the mRNA of XBP1, thereby generating an active isoform of this transcription factor...
  6. Plongthongkum N, Kullawong N, Panyim S, Tirasophon W. Ire1 regulated XBP1 mRNA splicing is essential for the unfolded protein response (UPR) in Drosophila melanogaster. Biochem Biophys Res Commun. 2007;354:789-94 pubmed
    ..Here, we report that Ire1p mediated XBP1 mRNA splicing mechanism is extremely conserved and exerts a critical role for modulating Xbp1 protein synthesis in ..
  7. Chang Y, Hung W, Chang Y, Chang H, Wu C, Chiang A, et al. Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model. PLoS Genet. 2011;7:e1001288 pubmed publisher
    ..Taken together, our analyses have defined the nature of IBMPFD-causing VCP mutations and made an unexpected link between cellular ATP level and IBMPFD pathogenesis...
  8. Kang M, Chung J, Ryoo H. CDK5 and MEKK1 mediate pro-apoptotic signalling following endoplasmic reticulum stress in an autosomal dominant retinitis pigmentosa model. Nat Cell Biol. 2012;14:409-15 pubmed publisher
    ..These findings establish a previously unrecognized branch of ER-stress response signalling involved in degenerative diseases. ..
  9. Zhang P, Zhou L, Pei C, Lin X, Yuan Z. Dysfunction of Wntless triggers the retrograde Golgi-to-ER transport of Wingless and induces ER stress. Sci Rep. 2016;6:19418 pubmed publisher
    ..Taken together, this study reveals a previously unknown Golgi-to-ER retrograde route of Wg, and elucidates a correlation between Wnt secretion and ER stress during development. ..
  10. O Sullivan N, Jahn T, Reid E, O Kane C. Reticulon-like-1, the Drosophila orthologue of the hereditary spastic paraplegia gene reticulon 2, is required for organization of endoplasmic reticulum and of distal motor axons. Hum Mol Genet. 2012;21:3356-65 pubmed publisher
    ..Our data provide a route to further understanding of both the role of the SER in axons and the pathological consequences of the impairment of this compartment. ..
  11. Velentzas P, Velentzas A, Mpakou V, Antonelou M, Margaritis L, Papassideri I, et al. Detrimental effects of proteasome inhibition activity in Drosophila melanogaster: implication of ER stress, autophagy, and apoptosis. Cell Biol Toxicol. 2013;29:13-37 pubmed publisher
    ..to result in the engagement of unfolded protein response (UPR) signaling pathway, as respectively indicated by fly Xbp1 activation and Ref(2)P-containing protein aggregate formation...
  12. Rincon Limas D, Jensen K, Fernandez Funez P. Drosophila models of proteinopathies: the little fly that could. Curr Pharm Des. 2012;18:1108-22 pubmed
    ..To remain relevant in this competitive environment, Drosophila models will need to expand their flexible, innovative, and multidisciplinary approaches to find new discoveries and translational applications. ..
  13. Rumpf S, Lee S, Jan L, Jan Y. Neuronal remodeling and apoptosis require VCP-dependent degradation of the apoptosis inhibitor DIAP1. Development. 2011;138:1153-60 pubmed publisher
    ..In cultured cells, VCP binds to DIAP1 in a ubiquitin- and BIR domain-dependent manner and facilitates its degradation. Our results establish a new link between ubiquitin, dendrite pruning and the apoptosis machinery. ..
  14. Marcora M, Belfiori Carrasco L, Bocai N, Morelli L, Castano E. Amyloid-?42 clearance and neuroprotection mediated by X-box binding protein 1 signaling decline with aging in the Drosophila brain. Neurobiol Aging. 2017;60:57-70 pubmed publisher
    ..that neuronal A?42 expression in Drosophila activated the inositol-requiring protein-1/X-box binding protein 1 (XBP1) UPR branch before the onset of behavioral impairment and persisted with aging...
  15. Nagy P, Varga A, Pircs K, Hegedűs K, Juhász G. Myc-driven overgrowth requires unfolded protein response-mediated induction of autophagy and antioxidant responses in Drosophila melanogaster. PLoS Genet. 2013;9:e1003664 pubmed publisher
    ..These novel results give additional support for finding future approaches to specifically inhibit the growth of cancer cells addicted to oncogenic Myc. ..
  16. Satoh T, Ohba A, Liu Z, Inagaki T, Satoh A. dPob/EMC is essential for biosynthesis of rhodopsin and other multi-pass membrane proteins in Drosophila photoreceptors. elife. 2015;4: pubmed publisher
    ..These results collectively indicate that EMC is a key factor in the biogenesis of multi-pass transmembrane proteins, including Rh1, and its loss causes retinal degeneration. ..
  17. Ryoo H. Drosophila as a model for unfolded protein response research. BMB Rep. 2015;48:445-53 pubmed
    ..The fact that the basic UPR pathways are conserved, together with the availability of many human disease models in this organism, makes Drosophila a powerful tool for studying human disease mechanisms. ..
  18. Maor G, Rencus Lazar S, Filocamo M, Steller H, Segal D, Horowitz M. Unfolded protein response in Gaucher disease: from human to Drosophila. Orphanet J Rare Dis. 2013;8:140 pubmed publisher
    ..Our results strongly suggest that mutant GCase induces the UPR in GD patients as well as in carriers of GD mutations and leads to development of locomotion deficit in flies heterozygous for GD mutations. ..
  19. Casso D, Liu S, Biehs B, Kornberg T. Expression and characterization of Drosophila signal peptide peptidase-like (sppL), a gene that encodes an intramembrane protease. PLoS ONE. 2012;7:e33827 pubmed publisher
    ..This was unexpected given that genetic knockdown phenotypes in other organisms suggested significant roles for Spp-related proteases. ..
  20. Pimenta de Castro I, Costa A, Lam D, Tufi R, Fedele V, Moisoi N, et al. Genetic analysis of mitochondrial protein misfolding in Drosophila melanogaster. Cell Death Differ. 2012;19:1308-16 pubmed publisher
    ..We show that in flies, a pathway involving pink1, parkin and ref(2)P has a role in the maintenance of a viable pool of cellular mitochondria by promoting organellar quality control. ..
  21. Loewen C, Feany M. The unfolded protein response protects from tau neurotoxicity in vivo. PLoS ONE. 2010;5: pubmed publisher
    ..We then use loss of function genetic reagents to support a role for the unfolded protein response in protecting from tau neurotoxicity. Our findings suggest that the unfolded protein response can ameliorate the toxicity of tau in vivo. ..
  22. Bhandari P, Song M, Dorn G. Dissociation of mitochondrial from sarcoplasmic reticular stress in Drosophila cardiomyopathy induced by molecularly distinct mitochondrial fusion defects. J Mol Cell Cardiol. 2015;80:71-80 pubmed publisher
    ..In contrast, enhancing the ability of endoplasmic/sarcoplasmic reticulum to handle stress by expressing Xbp1 rescued the cardiomyopathy of mitofusin/MARF insufficiency without improving that caused by Opa1 deficiency...
  23. Liang C, Chang Y, Chang H, Wang C, Hung Y, Lin Y, et al. Derlin-1 regulates mutant VCP-linked pathogenesis and endoplasmic reticulum stress-induced apoptosis. PLoS Genet. 2014;10:e1004675 pubmed publisher
    ..As this Derlin-1-dependent cell death is negated by TER94 overexpression, we propose that while Derlin-1 and VCP work cooperatively in ER stress response, their imbalance has a role in removing cells suffering prolonged ER stress. ..
  24. Coelho D, Domingos P. Physiological roles of regulated Ire1 dependent decay. Front Genet. 2014;5:76 pubmed publisher
    ..Activated Ire1 mediates the splicing of an intron from the mRNA of Xbp1, causing a frame-shift during translation and introducing a new carboxyl domain in the Xbp1 protein, which only ..
  25. Song Y, Sretavan D, Salegio E, Berg J, Huang X, Cheng T, et al. Regulation of axon regeneration by the RNA repair and splicing pathway. Nat Neurosci. 2015;18:817-25 pubmed publisher
    ..operate in the RNA repair and splicing pathway important for stress-induced mRNA splicing, including that of Xbp1, a cellular stress sensor...
  26. Haecker A, Bergman M, Neupert C, Moussian B, Luschnig S, Aebi M, et al. Wollknauel is required for embryo patterning and encodes the Drosophila ALG5 UDP-glucose:dolichyl-phosphate glucosyltransferase. Development. 2008;135:1745-9 pubmed publisher
    ..According to this view, inappropriate UPR activation can cause specific patterning defects during embryo development. ..
  27. Park S, Ludwig M, Tamarina N, He B, Carl S, Dickerson D, et al. Genetic complexity in a Drosophila model of diabetes-associated misfolded human proinsulin. Genetics. 2014;196:539-55 pubmed publisher
    ..Mutant (but not wild-type) proinsulin expression in the eye imaginal disc induces IRE1-mediated XBP1 alternative splicing, a signal for endoplasmic reticulum stress response activation, and produces global change in ..
  28. Liu Y, Lu W, BRUMMEL T, Yuh C, Lin P, Kao T, et al. Reduced expression of alpha-1,2-mannosidase I extends lifespan in Drosophila melanogaster and Caenorhabditis elegans. Aging Cell. 2009;8:370-9 pubmed publisher
    ..These findings suggested that mutations in mas1 may extend longevity by modulating DR. ..
  29. Lindström R, Lindholm P, Kallijärvi J, Palgi M, Saarma M, Heino T. Exploring the Conserved Role of MANF in the Unfolded Protein Response in Drosophila melanogaster. PLoS ONE. 2016;11:e0151550 pubmed publisher
    ..vivo approach we found genetic interactions between Drosophila Manf and genes encoding for Drosophila homologues of GRP78, PERK and XBP1, the key components of UPR. Our data suggest a role for Manf in the regulation of Drosophila UPR.
  30. Griciuc A, Aron L, Roux M, Klein R, Giangrande A, Ueffing M. Inactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in Drosophila. PLoS Genet. 2010;6: pubmed publisher
    ..Genetic inactivation of VCP increased the levels of misfolded Rh1(P37H) and further activated the Ire1/Xbp1 ER stress pathway in the Rh1(P37H) retina...
  31. Sánchez Alvarez M, Zhang Q, Finger F, Wakelam M, Bakal C. Cell cycle progression is an essential regulatory component of phospholipid metabolism and membrane homeostasis. Open Biol. 2015;5:150093 pubmed publisher
    ..We propose that coupling lipid metabolism to cell cycle progression is a means by which cells have evolved to coordinate proliferation with cell and organelle growth. ..
  32. Katz M, Acevedo J, Loenarz C, Galagovsky D, Liu Yi P, Pérez Pepe M, et al. Sudestada1, a Drosophila ribosomal prolyl-hydroxylase required for mRNA translation, cell homeostasis, and organ growth. Proc Natl Acad Sci U S A. 2014;111:4025-30 pubmed publisher
    ..These observations, together with those on enzyme homologs described in the companion articles, reveal conserved biochemical and biological roles for a widely distributed ribosomal oxygenase. ..
  33. Xu Z, Chikka M, Xia H, Ready D. Ire1 supports normal ER differentiation in developing Drosophila photoreceptors. J Cell Sci. 2016;129:921-9 pubmed publisher
    ..Instead, ER proliferates in persistent and ribosome-poor tubular tangles. A phase of Ire1 activity early in R cell development thus shapes dynamic ER. ..
  34. Wang L, Ryoo H, Qi Y, Jasper H. PERK Limits Drosophila Lifespan by Promoting Intestinal Stem Cell Proliferation in Response to ER Stress. PLoS Genet. 2015;11:e1005220 pubmed publisher
    ..Our studies highlight the significance of the PERK branch of the unfolded protein response of the ER (UPRER) in intestinal homeostasis and provide a viable strategy to improve organismal health- and lifespan. ..
  35. Wang Z, Rabouille C, Geisbrecht E. Loss of a Clueless-dGRASP complex results in ER stress and blocks Integrin exit from the perinuclear endoplasmic reticulum in Drosophila larval muscle. Biol Open. 2015;4:636-48 pubmed publisher
    ..This, in turn, is essential for integrin subunit αPS2 ER exit in Drosophila larval myofibers. ..
  36. Arsham A, Neufeld T. A genetic screen in Drosophila reveals novel cytoprotective functions of the autophagy-lysosome pathway. PLoS ONE. 2009;4:e6068 pubmed publisher
  37. Chow C, Avila F, Clark A, Wolfner M. Induction of excessive endoplasmic reticulum stress in the Drosophila male accessory gland results in infertility. PLoS ONE. 2015;10:e0119386 pubmed publisher
    ..Both genetic manipulations induced excessive ER stress in the AG, as indicated by the increase in Xbp1 splicing, a marker of ER stress...
  38. Coelho D, Gaspar C, Domingos P. Ire1 mediated mRNA splicing in a C-terminus deletion mutant of Drosophila Xbp1. PLoS ONE. 2014;9:e105588 pubmed publisher
    ..ER stress, promotes the unconventional splicing of a small intron from the mRNA encoding the transcription factor Xbp1. In mammals, two specific regions (the hydrophobic region 2--HR2--and the C-terminal translational pausing site) ..
  39. Lin M, Carlson J, Crosby M, Matthews B, Yu C, Park S, et al. Revisiting the protein-coding gene catalog of Drosophila melanogaster using 12 fly genomes. Genome Res. 2007;17:1823-36 pubmed
    ..These results affect >10% of annotated fly genes and demonstrate the power of comparative genomics to enhance our understanding of genome organization, even in a model organism as intensively studied as Drosophila melanogaster. ..
  40. Kang K, Ryoo H, Park J, Yoon J, Kang M. A Drosophila Reporter for the Translational Activation of ATF4 Marks Stressed Cells during Development. PLoS ONE. 2015;10:e0126795 pubmed publisher
    ..These results establish a new tool to study ATF4-mediated stress response in Drosophila development and disease. ..
  41. Baranov P, Wills N, Barriscale K, Firth A, Jud M, Letsou A, et al. Programmed ribosomal frameshifting in the expression of the regulator of intestinal stem cell proliferation, adenomatous polyposis coli (APC). RNA Biol. 2011;8:637-47 pubmed publisher
    ..Three other proposed PRF candidates (Xbp1, CG32736, CG14047) with switches in conservation of reading frames are likely explained by mechanisms other than ..
  42. Yamakawa T, Yamada K, Sasamura T, Nakazawa N, Kanai M, Suzuki E, et al. Deficient Notch signaling associated with neurogenic pecanex is compensated for by the unfolded protein response in Drosophila. Development. 2012;139:558-67 pubmed publisher
    ..In addition, hyper-induction of the unfolded protein response (UPR) by the expression of activated Xbp1 or dominant-negative Heat shock protein cognate 3 suppressed the neurogenic phenotype and ER enlargement caused by ..
  43. Johansson A, Stenberg P, Bernhardsson C, Larsson J. Painting of fourth and chromosome-wide regulation of the 4th chromosome in Drosophila melanogaster. EMBO J. 2007;26:2307-16 pubmed
    ..We propose a balancing mechanism involving POF and HP1 that provides a feedback system for fine-tuning expression status of genes on the 4th chromosome. ..
  44. Groth C, Sasamura T, Khanna M, Whitley M, Fortini M. Protein trafficking abnormalities in Drosophila tissues with impaired activity of the ZIP7 zinc transporter Catsup. Development. 2013;140:3018-27 pubmed publisher
    ..In addition, Catsup mutant cells exhibit elevated ER stress, suggesting that impaired zinc homeostasis causes increased levels of misfolded proteins within the secretory compartment. ..
  45. Wang L, Zeng X, Ryoo H, Jasper H. Integration of UPRER and oxidative stress signaling in the control of intestinal stem cell proliferation. PLoS Genet. 2014;10:e1004568 pubmed publisher
    ..Our results identify a signaling network of proteostatic and oxidative stress responses that regulates ISC function and regenerative homeostasis in the intestinal epithelium. ..
  46. Debattisti V, Pendin D, Ziviani E, Daga A, Scorrano L. Reduction of endoplasmic reticulum stress attenuates the defects caused by Drosophila mitofusin depletion. J Cell Biol. 2014;204:303-12 pubmed publisher
    ..Thus, ER stress is a targetable pathogenetic component of the phenotypes caused by Drosophila Mfn ablation. ..
  47. Malzer E, Szajewska Skuta M, Dalton L, Thomas S, Hu N, Skaer H, et al. Coordinate regulation of eIF2α phosphorylation by PPP1R15 and GCN2 is required during Drosophila development. J Cell Sci. 2013;126:1406-15 pubmed publisher
    ..Moreover, we show that its expression is necessary for embryonic and larval development and that this is to oppose the inhibitory effects of GCN2 on anabolic growth. ..