Gene Symbol: DnaJ-1
Description: DnaJ-like-1
Alias: CG10578, DNAJ-1, DNAJ1, DROJ1, DmHsp40, Dmel\CG10578, DnaJ1, DnaJ1 64EF, DroJ1, EU3500, HSP40, HSP40/HDJ1, Hdj1, Hsp-40, Hsp40, anon-WO0140519.166, anon-WO0172774.135, dHDJ1, dHDJ1/HSP40, dHdj1, dhdJ1, dhdj-1, dhdj1, dnaJ-1, droj1, hsp40, DnaJ-like-1, CG10578-PA, CG10578-PB, DnaJ-1-PA, DnaJ-1-PB, Hsp40, dnaJ like1
Species: fruit fly

Top Publications

  1. Cobreros L, Fernández Miñán A, Luque C, González Reyes A, Martin Bermudo M. A role for the chaperone Hsp70 in the regulation of border cell migration in the Drosophila ovary. Mech Dev. 2008;125:1048-58 pubmed publisher
    ..Similar defects are found when the Hsp70 co-chaperone DnaJ-1, the Drosophila homolog of the human Hsp40, is overexpressed specifically in BCs...
  2. Fernandez Funez P, Nino Rosales M, de Gouyon B, She W, Luchak J, Martinez P, et al. Identification of genes that modify ataxin-1-induced neurodegeneration. Nature. 2000;408:101-6 pubmed
    ..These findings may be relevant to the treatment of polyglutamine diseases and, perhaps, to other neurodegenerative diseases, such as Alzheimer's and Parkinson's disease. ..
  3. Bilen J, Bonini N. Genome-wide screen for modifiers of ataxin-3 neurodegeneration in Drosophila. PLoS Genet. 2007;3:1950-64 pubmed
    ..These findings provide new insight into molecular pathways of polyQ toxicity, defining novel targets for promoting neuronal survival in human neurodegenerative disease. ..
  4. Marsh J, Thompson L. Can flies help humans treat neurodegenerative diseases?. Bioessays. 2004;26:485-96 pubmed
  5. Fayazi Z, Ghosh S, Marion S, Bao X, Shero M, Kazemi Esfarjani P. A Drosophila ortholog of the human MRJ modulates polyglutamine toxicity and aggregation. Neurobiol Dis. 2006;24:226-44 pubmed
    ..In the photoreceptors, expression of another suppressor with a J domain, dHDJ1, but not dMRJ, prior to expression of expanded polyQs dramatically promoted cytoplasmic aggregation...
  6. Takeyama K, Ito S, Yamamoto A, Tanimoto H, Furutani T, Kanuka H, et al. Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila. Neuron. 2002;35:855-64 pubmed
    ..Thus, our findings suggest that the full binding of androgen to the polyQ-expanded hAR mutants leads to structural alteration with nuclear translocation that eventually results in the onset of SBMA in male patients. ..
  7. Ghosh S, Feany M. Comparison of pathways controlling toxicity in the eye and brain in Drosophila models of human neurodegenerative diseases. Hum Mol Genet. 2004;13:2011-8 pubmed
    ..These findings outline common pathways of neurotoxicity, demonstrate disease- and cell-type specific pathways and identify a common vitamin as a potential therapy in polyglutamine disorders. ..
  8. Chan H, Warrick J, Gray Board G, Paulson H, Bonini N. Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila. Hum Mol Genet. 2000;9:2811-20 pubmed
    ..These findings have implications for these and other human neurodegenerative diseases associated with abnormal protein aggregation. ..
  9. Doumanis J, Wada K, Kino Y, Moore A, Nukina N. RNAi screening in Drosophila cells identifies new modifiers of mutant huntingtin aggregation. PLoS ONE. 2009;4:e7275 pubmed publisher
    ..Newly identified modifiers including genes related to nuclear transport, nucleotide processes, and signaling, may be involved in polyglutamine aggregate formation and Huntington disease cascades. ..

More Information


  1. Landis G, Abdueva D, Skvortsov D, Yang J, Rabin B, Carrick J, et al. Similar gene expression patterns characterize aging and oxidative stress in Drosophila melanogaster. Proc Natl Acad Sci U S A. 2004;101:7663-8 pubmed
    ..Immune reporter expression in young flies was partially predictive of remaining life span, suggesting their potential as biomonitors of aging. ..
  2. Marchler G, Wu C. Modulation of Drosophila heat shock transcription factor activity by the molecular chaperone DROJ1. EMBO J. 2001;20:499-509 pubmed
    ..DROJ1, the fly counterpart of the human heat shock protein HSP40/HDJ1, was identified as the dominant interacting protein (15 independent isolates from 58 candidates)...
  3. Kazemi Esfarjani P, Benzer S. Genetic suppression of polyglutamine toxicity in Drosophila. Science. 2000;287:1837-40 pubmed
    ..The predicted product of one, dHDJ1, is homologous to human heat shock protein 40/HDJ1...
  4. Fujikake N, Nagai Y, Popiel H, Okamoto Y, Yamaguchi M, Toda T. Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones. J Biol Chem. 2008;283:26188-97 pubmed publisher
    ..3% rescue), indicating that 17-AAG is widely effective against various polyQ diseases. 17-AAG induced Hsp70, Hsp40, and Hsp90 expression in a dose-dependent manner, and the expression levels correlated with its therapeutic ..
  5. Shaw P, Tononi G, Greenspan R, Robinson D. Stress response genes protect against lethal effects of sleep deprivation in Drosophila. Nature. 2002;417:287-91 pubmed
    ..These data represent the first step in identifying the molecular mechanisms that constitute the sleep homeostat. ..
  6. Colinet H, Siaussat D, Bozzolan F, Bowler K. Rapid decline of cold tolerance at young age is associated with expression of stress genes in Drosophila melanogaster. J Exp Biol. 2013;216:253-9 pubmed publisher
    ..We also assessed whether expression (basal and induced) of eight stress genes (hsp22, hsp23, hsp40, hsp68, hsp70Aa, hsp83, Starvin and Frost) varied post-eclosion in correspondence with changes found in cold ..
  7. Gonsalves S, Moses A, Razak Z, Robert F, Westwood J. Whole-genome analysis reveals that active heat shock factor binding sites are mostly associated with non-heat shock genes in Drosophila melanogaster. PLoS ONE. 2011;6:e15934 pubmed publisher
    ..These results suggest that Drosophila HSF may be regulating many genes besides the known HS genes and that some of these genes may be regulated during non-stress conditions. ..
  8. Demontis F, Perrimon N. FOXO/4E-BP signaling in Drosophila muscles regulates organism-wide proteostasis during aging. Cell. 2010;143:813-25 pubmed publisher
    ..These findings reveal an organism-wide regulation of proteostasis in response to muscle aging and a key role of FOXO/4E-BP signaling in the coordination of organismal and tissue aging. ..
  9. Nishimura Y, Yalgin C, Akimoto S, Doumanis J, Sasajima R, Nukina N, et al. Selection of behaviors and segmental coordination during larval locomotion is disrupted by nuclear polyglutamine inclusions in a new Drosophila Huntington's disease-like model. J Neurogenet. 2010;24:194-206 pubmed publisher
    ..From these analyses the authors show that examination of motor behaviors in the Drosophila larvae is a powerful new model to dissect non-cell-lethal mechanisms of mutant Htt toxicity. ..
  10. Simões Correia J, Silva D, Melo S, Figueiredo J, Caldeira J, Pinto M, et al. DNAJB4 molecular chaperone distinguishes WT from mutant E-cadherin, determining their fate in vitro and in vivo. Hum Mol Genet. 2014;23:2094-105 pubmed publisher
    ..DnaJ (Hsp40) homolog, subfamily B, member 4 (DNAJB4), the human homolog of DnaJ-1, influences Ecad localization and stability ..
  11. 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. ..
  12. Colinet H, Lee S, Hoffmann A. Temporal expression of heat shock genes during cold stress and recovery from chill coma in adult Drosophila melanogaster. FEBS J. 2010;277:174-85 pubmed publisher
    ..response, we consider that Hsp60, Hsp67Ba and Hsc70-1 are not cold-inducible, whereas Hsp22, Hsp23, Hsp26, Hsp27, Hsp40, Hsp68, Hsp70Aa and Hsp83 are induced by cold...
  13. Bettencourt B, Hogan C, Nimali M, Drohan B. Inducible and constitutive heat shock gene expression responds to modification of Hsp70 copy number in Drosophila melanogaster but does not compensate for loss of thermotolerance in Hsp70 null flies. BMC Biol. 2008;6:5 pubmed publisher
  14. Tsou W, Hosking R, Burr A, Sutton J, Ouyang M, Du X, et al. DnaJ-1 and karyopherin α3 suppress degeneration in a new Drosophila model of Spinocerebellar Ataxia Type 6. Hum Mol Genet. 2015;24:4385-96 pubmed publisher
    ..Invertebrate animal models of SCA6 can expand our understanding of molecular sequelae related to degeneration in this disorder and lead to the rapid identification of cellular components that can be targeted to treat it. ..
  15. Wang L, Zhou L, Zhu Z, Ma W, Lei C. Differential temporal expression profiles of heat shock protein genes in Drosophila melanogaster (Diptera: Drosophilidae) under ultraviolet A radiation stress. Environ Entomol. 2014;43:1427-34 pubmed publisher
    ..So DmHsps might act in a coordinated and cooperative manner at the transcriptional level to counteract UV-A radiation-based stress. ..
  16. Sinadinos C, Quraishe S, Sealey M, Samson P, Mudher A, Wyttenbach A. Low endogenous and chemical induced heat shock protein induction in a 0N3Rtau-expressing Drosophila larval model of Alzheimer's disease. J Alzheimers Dis. 2013;33:1117-33 pubmed publisher
    ..Forthcoming studies investigating the effects of HSP induction on tau-mediated dysfunction/toxicity in such models will require more robust, non-pharmacological (perhaps genetic) means of manipulating the hsp90 pathway. ..
  17. Sutton J, Blount J, Libohova K, Tsou W, Joshi G, Paulson H, et al. Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3. Hum Mol Genet. 2017;26:1419-1431 pubmed publisher
  18. Štětina T, Koštál V, Korbelová J. The Role of Inducible Hsp70, and Other Heat Shock Proteins, in Adaptive Complex of Cold Tolerance of the Fruit Fly (Drosophila melanogaster). PLoS ONE. 2015;10:e0128976 pubmed publisher
    ..Genetic elimination of Hsp70 up-regulation response has no effect on survival of chronic exposures to 0°C or mild acute cold shocks, while it negatively affects survival after severe acute cold shocks at temperatures below -8°C. ..
  19. Liévens J, Iché M, Laval M, Faivre Sarrailh C, Birman S. AKT-sensitive or insensitive pathways of toxicity in glial cells and neurons in Drosophila models of Huntington's disease. Hum Mol Genet. 2008;17:882-94 pubmed
    ..ERK had no beneficial effects in the retina or brain. These results indicate that mHtt activates distinct pathways of toxicity in Drosophila, either sensitive to AKT in retinal photoreceptors and glia, or independent in brain neurons. ..
  20. Tsou W, Ouyang M, Hosking R, Sutton J, Blount J, Burr A, et al. The deubiquitinase ataxin-3 requires Rad23 and DnaJ-1 for its neuroprotective role in Drosophila melanogaster. Neurobiol Dis. 2015;82:12-21 pubmed publisher
    ..Through DnaJ-1, our data connect ataxin-3 and Rad23 to protective processes involved with protein folding rather than increased turnover of toxic polyQ species. ..
  21. Takeuchi T, Suzuki M, Fujikake N, Popiel H, Kikuchi H, Futaki S, et al. Intercellular chaperone transmission via exosomes contributes to maintenance of protein homeostasis at the organismal level. Proc Natl Acad Sci U S A. 2015;112:E2497-506 pubmed publisher
    ..We found that elevated expression of molecular chaperones, such as Hsp40 and Hsp70, in a group of cells improves proteostasis in other groups of cells, both in cultured cells and in ..
  22. Reinhardt A, Feuillette S, Cassar M, Callens C, Thomassin H, Birman S, et al. Lack of miRNA Misregulation at Early Pathological Stages in Drosophila Neurodegenerative Disease Models. Front Genet. 2012;3:226 pubmed publisher
    ..Thus our data suggest that transcriptional deregulation of miRNAs or sCAG is unlikely to play a significant role in the initial stages of neurodegenerative diseases. ..
  23. Garbuz D, Molodtsov V, Velikodvorskaia V, Evgen ev M, Zatsepina O. [Evolution of the response to heat shock in genus Drosophila]. Genetika. 2002;38:1097-109 pubmed
    ..The results obtained suggest that the HSP40 and low-molecular-weight HSPs (lmwHSPs) play a significant role in thermotolerance and adaptation to hot climate...
  24. MILLER M, Chen A, Gobert V, Augé B, Beau M, Burlet Schiltz O, et al. Control of RUNX-induced repression of Notch signaling by MLF and its partner DnaJ-1 during Drosophila hematopoiesis. PLoS Genet. 2017;13:e1006932 pubmed publisher
    ..Our results show that MLF and the Hsp40 co-chaperone family member DnaJ-1 interact through conserved domains and we demonstrate that both proteins bind ..
  25. Shaw P, Franken P. Perchance to dream: solving the mystery of sleep through genetic analysis. J Neurobiol. 2003;54:179-202 pubmed
    ..In this article we will review recent studies that have used genetic techniques to evaluate sleep in the fruit fly and the mouse. ..
  26. Carmel J, Rashkovetsky E, Nevo E, Korol A. Differential expression of small heat shock protein genes Hsp23 and Hsp40, and heat shock gene Hsr-omega in fruit flies (Drosophila melanogaster) along a microclimatic gradient. J Hered. 2011;102:593-603 pubmed publisher
    We examined the role of small Hsp genes (Hsp23 and Hsp40) and heat shock gene Hsr-omega in the thermoadaptation of Drosophila melanogaster inhabiting a highly heterogeneous microsite (Nahal Oren canyon, Carmel massif, Israel)...
  27. Tian S, Haney R, Feder M. Phylogeny disambiguates the evolution of heat-shock cis-regulatory elements in Drosophila. PLoS ONE. 2010;5:e10669 pubmed publisher
    ..The broad dimensions of variation uncovered are particularly important as they suggest a substantial challenge for functional studies. ..
  28. Nisoli I, Chauvin J, Napoletano F, Calamita P, Zanin V, Fanto M, et al. Neurodegeneration by polyglutamine Atrophin is not rescued by induction of autophagy. Cell Death Differ. 2010;17:1577-87 pubmed publisher
    ..Our data thus provide a crucial insight into the specific mechanism of a polyglutamine disease and reveal important differences in the role of autophagy with respect to other diseases of the same family. ..
  29. Wang C, Chen Y, Wang Y, Huang M, Yen T, Li H, et al. Reduced neuronal expression of ribose-5-phosphate isomerase enhances tolerance to oxidative stress, extends lifespan, and attenuates polyglutamine toxicity in Drosophila. Aging Cell. 2012;11:93-103 pubmed publisher
  30. Sessions O, Barrows N, Souza Neto J, Robinson T, Hershey C, Rodgers M, et al. Discovery of insect and human dengue virus host factors. Nature. 2009;458:1047-50 pubmed publisher
    ..This indicates notable conservation of required factors between dipteran and human hosts. This work suggests new approaches to control infection in the insect vector and the mammalian host. ..
  31. Kuo Y, Ren S, Lao U, Edgar B, Wang T. Suppression of polyglutamine protein toxicity by co-expression of a heat-shock protein 40 and a heat-shock protein 110. Cell Death Dis. 2013;4:e833 pubmed publisher
    ..In a Drosophila model of polyglutamine (polyQ) disease, the HSP40 family protein, DNAJ-1, is a superior suppressor of toxicity caused by the aggregation of polyQ containing ..
  32. Bonini N. Chaperoning brain degeneration. Proc Natl Acad Sci U S A. 2002;99 Suppl 4:16407-11 pubmed
    ..Hsp70 is a potent suppressor of both polyglutamine disease and Parkinson's disease in Drosophila. These studies provide the promise of treatments for human neurodegeneration through the up-regulation of stress and chaperone pathways. ..
  33. Tsai C, Kao H, Mitzutani A, Banayo E, Rajan H, McKeown M, et al. Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors. Proc Natl Acad Sci U S A. 2004;101:4047-52 pubmed
    ..Together, our results suggest that Atx1 is a transcriptional factor whose mutant form exerts its deleterious effects in part by perturbing corepressor-dependent transcriptional pathways. ..
  34. Chen X, Burgoyne R. Identification of common genetic modifiers of neurodegenerative diseases from an integrative analysis of diverse genetic screens in model organisms. BMC Genomics. 2012;13:71 pubmed publisher
  35. Na D, Rouf M, O Kane C, Rubinsztein D, Gsponer J. NeuroGeM, a knowledgebase of genetic modifiers in neurodegenerative diseases. BMC Med Genomics. 2013;6:52 pubmed publisher
    ..As the first compendium of genetic modifiers, NeuroGeM will assist experimental and computational scientists in their search for the pathophysiological mechanisms underlying NDs. http://chibi.ubc.ca/neurogem. ..
  36. Chartier A, Benoit B, Simonelig M. A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1. EMBO J. 2006;25:2253-62 pubmed
    ..We also identify several suppressors of the OPMD phenotype. This establishes our OPMD Drosophila model as a powerful in vivo test to understand the disease process and develop novel therapeutic strategies...
  37. Korol A, Rashkovetsky E, Iliadi K, Nevo E. Drosophila flies in "Evolution Canyon" as a model for incipient sympatric speciation. Proc Natl Acad Sci U S A. 2006;103:18184-9 pubmed
    ..We suggest that Drosophila populations at EC represent a rare example, demonstrating how selection overrides migration, and propose an ad hoc ecological model of incipient sympatric divergence. ..