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Abnormal interaction of motor neuropathy-associated mutant HspB8 (Hsp22) forms with the RNA helicase Ddx20 (gemin3)

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Cell Stress and Chaperones Aims and scope

Abstract

A number of missense mutations in the two related small heat shock proteins HspB8 (Hsp22) and HspB1 (Hsp27) have been associated with the inherited motor neuron diseases (MND) distal hereditary motor neuropathy and Charcot-Marie-Tooth disease. HspB8 and HspB1 interact with each other, suggesting that these two etiologic factors may act through a common biochemical mechanism. However, their role in neuron biology and in MND is not understood. In a yeast two-hybrid screen, we identified the DEAD box protein Ddx20 (gemin3, DP103) as interacting partner of HspB8. Using co-immunoprecipitation, chemical cross-linking, and in vivo quantitative fluorescence resonance energy transfer, we confirmed this interaction. We also show that the two disease-associated mutant HspB8 forms have abnormally increased binding to Ddx20. Ddx20 itself binds to the survival-of-motor-neurons protein (SMN protein), and mutations in the SMN1 gene cause spinal muscular atrophy, another MND and one of the most prevalent genetic causes of infant mortality. Thus, these protein interaction data have linked the three etiologic factors HspB8, HspB1, and SMN protein, and mutations in any of their genes cause the various forms of MND. Ddx20 and SMN protein are involved in spliceosome assembly and pre-mRNA processing. RNase treatment affected the interaction of the mutant HspB8 with Ddx20 suggesting RNA involvement in this interaction and a potential role of HspB8 in ribonucleoprotein processing.

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References

  • Ackerley S, James PA, Kalli A, French S, Davies KE, Talbot KA (2006) A mutation in the small heat-shock protein HSPB1 leading to distal hereditary motor neuronopathy disrupts neurofilament assembly and the axonal transport of specific cellular cargoes. Hum Mol Genet 15:347–354

    Article  CAS  PubMed  Google Scholar 

  • Badri KR, Modem S, Gerard HC, Khan I, Bagchi M, Hudson AP, Reddy TR (2006) Regulation of Sam68 activity by small heat shock protein 22. J Cell Biochem 99:1353–1362

    Article  CAS  PubMed  Google Scholar 

  • Benn SC, Perrelet D, Kato AC, Scholz J, Decosterd I, Mannion RJ, Bakowska JC, Woolf CJ (2002) Hsp27 upregulation and phosphorylation is required for injured sensory and motor neuron survival. Neuron 36:45–56

    Article  CAS  PubMed  Google Scholar 

  • Benndorf R (2010) HspB1 and HspB8 Mutations in Neuropathies. In: Simon S, Arrigo A-P (eds) Small stress proteins in human diseases. Nova Publishers (NY) (in press)

  • Benndorf R, Welsh MJ (2004) Shocking degeneration. Nat Genet 36:547–548

    Article  CAS  PubMed  Google Scholar 

  • Benndorf R, Engel K, Gaestel M (2000) Analysis of small Hsp phosphorylation. Methods Mol Biol 99:431–434

    CAS  PubMed  Google Scholar 

  • Benndorf R, Sun X, Gilmont RR, Biederman KJ, Molloy MP, Goodmurphy CW, Cheng H, Andrews PC, Welsh MJ (2001) HSP22, a new member of the small heat shock protein superfamily, interacts with mimic of phosphorylated HSP27 (3DHSP27). J Biol Chem 276:26753–26761

    Article  CAS  PubMed  Google Scholar 

  • Bruey JM, Ducasse C, Bonniaud P, Ravagnan L, Susin SA, Diaz-Latoud C, Gurbuxani S, Arrigo AP, Kroemer G, Solary E, Garrido C (2000) Hsp27 negatively regulates cell death by interacting with cytochrome c. Nat Cell Biol 2:645–652

    Article  CAS  PubMed  Google Scholar 

  • Bryantsev AL, Chechenova MB, Shelden EA (2007) Recruitment of phosphorylated small heat shock protein Hsp27 to nuclear speckles without stress. Exp Cell Res 313:195–209

    Article  CAS  PubMed  Google Scholar 

  • Carra S, Sivilotti M, Chavez-Zobel AT, Lambert H, Landry J (2005) HspB8, a small heat shock protein mutated in human neuromuscular disorders, has in vivo chaperone activity in cultured cells. Hum Mol Genet 14:1659–1669

    Article  CAS  PubMed  Google Scholar 

  • Carra S, Seguin SJ, Lambert H, Landry J (2008) HspB8 chaperone activity toward poly(Q)-containing proteins depends on its association with Bag3, a stimulator of macroautophagy. J Biol Chem 283:1437–1444

    Article  CAS  PubMed  Google Scholar 

  • Carra S, Brunsting JF, Lambert H, Landry J, Kampinga HH (2009) HspB8 participates in protein quality control by a non-chaperone-like mechanism that requires eIF2α phosphorylation. J Biol Chem 284:5523–5532

    Article  CAS  PubMed  Google Scholar 

  • Charroux B, Pellizzoni L, Perkinson RA, Shevchenko A, Mann M, Dreyfuss G (1999) Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems. J Cell Biol 147:1181–1193

    Article  CAS  PubMed  Google Scholar 

  • Chávez-Zobel AT, Loranger A, Marceau N, Thériault JR, Lambert H, Landry J (2003) Distinct chaperone mechanisms can delay the formation of aggresomes by the myopathy-causing R120G αB-crystallin mutant. Hum Hum Mol Genet 12:1609–1620

    Article  Google Scholar 

  • Chowdary TK, Raman B, Ramakrishna T, Rao CM (2004) Mammalian Hsp22 is a heat-inducible small heat-shock protein with chaperone-like activity. Biochem J 381(Pt2):379–387

    CAS  PubMed  Google Scholar 

  • Cuesta R, Laroia G, Schneider RJ (2000) Chaperone hsp27 inhibits translation during heat shock by binding eIF4G and facilitating dissociation of cap-initiation complexes. Genes Dev 14:1460–1470

    CAS  PubMed  Google Scholar 

  • den Engelsman J, Gerrits D, de Jong WW, Robbins J, Kato K, Boelens WC (2005) Nuclear import of αB-crystallin is phosphorylation-dependent and hampered by hyperphosphorylation of the myopathy-related mutant R120G. J Biol Chem 280:37139–37148

    Article  Google Scholar 

  • Dierick I, Irobi J, De Jonghe P, Timmerman V (2005) Small heat shock proteins in inherited peripheral neuropathies. Ann Med 37:413–422

    Article  CAS  PubMed  Google Scholar 

  • Donker RB, Mouillet JF, Nelson DM, Sadovsky Y (2007) The expression of Argonaute2 and related microRNA biogenesis proteins in normal and hypoxic trophoblasts. Mol Hum Reprod 13:273–279

    Article  CAS  PubMed  Google Scholar 

  • Fontaine J-M, Rest JS, Welsh MJ, Benndorf R (2003) The sperm outer dense fiber protein is the 10th member of the superfamily of mammalian small stress proteins. Cell Stress Chaperones 8:62–69

    Article  CAS  PubMed  Google Scholar 

  • Fontaine J-M, Sun X, Benndorf R, Welsh MJ (2005) Interactions of HSP22 with HSP20, αB-crystallin, and HSPB3. Biochem Biophys Res Commun 337:1006–1011

    Article  CAS  PubMed  Google Scholar 

  • Fontaine J-M, Sun X, Hoppe AD, Simon S, Vicart P, Welsh MJ, Benndorf R (2006) Abnormal small heat shock protein interactions involving neuropathy-associated HSP22 (HSPB8) mutants. FASEB J 20:2168–2170

    Article  CAS  PubMed  Google Scholar 

  • Fuller-Pace FV (2006) DExD/H box RNA helicases: multifunctional proteins with important roles in transcriptional regulation. Nucleic Acids Res 34:4206–4215

    Article  CAS  PubMed  Google Scholar 

  • Fuller-Pace FV, Jacobs AM, Nicol SM (2007) Modulation of transcriptional activity of the DEAD-box family of RNA helicases, p68 (Ddx5) and DP103 (Ddx20), by SUMO modification. Biochem Soc Trans 35(Pt6):1427–1429

    Article  CAS  PubMed  Google Scholar 

  • Gillian AL, Svaren J (2004) The Ddx20/DP103 dead box protein represses transcriptional activation by Egr2/Krox-20. J Biol Chem 279:9056–9063

    Article  CAS  PubMed  Google Scholar 

  • Grundhoff AT, Kremmer E, Türeci O, Glieden A, Gindorf C, Atz J, Mueller-Lantzsch N, Schubach WH, Grässer FA (1999) Characterization of DP103, a novel DEAD box protein that binds to the Epstein-Barr virus nuclear proteins EBNA2 and EBNA3C. J Biol Chem 274:19136–19144

    Article  CAS  PubMed  Google Scholar 

  • Gubitz AK, Feng W, Dreyfuss G (2004) The SMN complex. Exp Cell Res 296:51–56

    Article  CAS  PubMed  Google Scholar 

  • Harding AE, Thomas PK (1980) The clinical features of hereditary motor and sensory neuropathy types I and II. Brain 103:259–280

    Article  CAS  PubMed  Google Scholar 

  • Hase M, Depre C, Vatner SF, Sadoshima J (2005) H11 has dose-dependent and dual hypertrophic and proapoptotic functions in cardiac myocytes. Biochem J 388:475–483

    Article  CAS  PubMed  Google Scholar 

  • Hedhli N, Wang L, Wang Q, Rashed E, Tian Y, Sui X, Madura K, Depre C (2008) Proteasome activation during cardiac hypertrophy by the chaperone H11 Kinase/Hsp22. Cardiovasc Res 77:497–505

    Article  CAS  PubMed  Google Scholar 

  • Hoppe A, Christensen K, Swanson JA (2002) Fluorescence resonance energy transfer-based stoichiometry in living cells. Biophys J 83:3652–3664

    Article  CAS  PubMed  Google Scholar 

  • Inagaki N, Hayashi T, Arimura T, Koga Y, Takahashi M, Shibata H, Teraoka K, Chikamori T, Yamashina A, Kimura A (2006) αB-crystallin mutation in dilated cardiomyopathy. Biochem Biophys Res Commun 342:379–386

    Article  CAS  PubMed  Google Scholar 

  • Inaguma Y, Hasegawa K, Goto S, Ito H, Kato K (1995) Induction of the synthesis of hsp27 and αB- crystallin in tissues of heat-stressed rats and its suppression by ethanol or an alpha 1-adrenergic antagonist. J Biochem 117:1238–1243

    CAS  PubMed  Google Scholar 

  • Irobi J, De Jonghe P, Timmerman V (2004a) Molecular genetics of distal hereditary motor neuropathies. Hum Mol Genet 13(Suppl 2):R195–R202

    Article  CAS  PubMed  Google Scholar 

  • Irobi J, Van Impe K, Seeman P, Jordanova A, Dierick I, Verpoorten N, Michalik A, De Vriendt E, Jacobs A, Van Gerwen V, Vennekens K, Mazanec R, Tournev I, Hilton-Jones D, Talbot K, Kremensky I, Van Den Bosch L, Robberecht W, Van Vandekerckhove J, Broeckhoven C, Gettemans J, De Jonghe P, Timmerman V (2004b) Hot-spot residue in small heat-shock protein 22 causes distal motor neuropathy. Nat Genet 36:597–601

    Article  CAS  PubMed  Google Scholar 

  • Irobi-Devolder J, Krishnan J, Almeida-Souza L-SC, Dierick I, Ceuterick-de Groote C, van den Bosch L, Timmermans J, Robberecht W, de Jonghe P, Janssens S, Timmerman VL. Mutant heat shock protein HSPB8 induces aggregation and a pro-apoptotic phenotype in distal motor neuropathy. 38th Annual Meeting of the Society for Neuroscience, Washington, DC, Nov. 15–19, 2008.

  • Jablonka S, Wiese S, Sendtner M (2004) Axonal defects in mouse models of motoneuron disease. J Neurobiol 58:272–286

    Article  CAS  PubMed  Google Scholar 

  • Jankowsky E, Jankowsky A (2000) The DExH/D protein family data base. Nucleic Acid Res 28:333–334

    Article  CAS  PubMed  Google Scholar 

  • Jia Y, Ransom RF, Shibanuma M, Liu C, Welsh MJ, Smoyer WE (2001) Identification and characterization of hic-5/ARA55 as an hsp27 binding protein. J Biol Chem 276:39911–39918

    Article  CAS  PubMed  Google Scholar 

  • Kappé G, Franck E, Verschuure P, Boelens WC, Leunissen JA, de Jong WW (2003) The human genome encodes 10 α-crystallin-related small heat shock proteins: HspB1-10. Cell Stress Chaperones 8:53–61

    Article  PubMed  Google Scholar 

  • Kappé G, Boelens WC, de Jong, WW (2010) Why proteins without an a-crystallin domain should not be included in the human small heat shock protein family HSPB. Cell Stress Chaperones, (in press)

  • Kasakov AS, Bukach OV, Seit-Nebi AS, Marston SB, Gusev NB (2007) Effect of mutations in the beta5-beta7 loop on the structure and properties of human small heat shock protein HSP22 (HspB8, H11). FEBS J 274:5628–5642

    Article  CAS  PubMed  Google Scholar 

  • Kim MV, Seit-Nebi AS, Marston SB, Gusev NB (2004) Some properties of human small heat shock protein Hsp22 (H11 or HspB8). Biochem Biophys Res Commun 315:796–801

    Article  CAS  PubMed  Google Scholar 

  • Kim MV, Kasakov AS, Seit-Nebi AS, Marston SB, Gusev NB (2006) Structure and properties of K141E mutant of small heat shock protein HSP22 (HspB8, H11) that is expressed in human neuromuscular disorders. Arch Biochem Biophys 454:32–41

    Article  CAS  PubMed  Google Scholar 

  • Klappacher GW, Lunyak VV, Sykes DB, Sawka-Verhelle D, Sage J, Brard G, Ngo SD, Gangadharan D, Jacks T, Kamps MP, Rose DW, Rosenfeld MG, Glass CK (2002) An induced Ets repressor complex regulates growth arrest during terminal macrophage differentiation. Cell 109:169–180

    Article  CAS  PubMed  Google Scholar 

  • Lasa M, Mahtani KR, Finch A, Brewer G, Saklatvala J, Clark AR (2000) Regulation of cyclooxygenase 2 mRNA stability by the mitogen-activated protein kinase p38 signaling cascade. Mol Cell Biol 20:4265–4274

    Article  CAS  PubMed  Google Scholar 

  • Lee MB, Lebedeva LA, Suzawa M, Wadekar SA, Desclozeaux M, Ingraham HA (2005a) The DEAD-box protein DP103 (Ddx20 or Gemin-3) represses orphan nuclear receptor activity via SUMO modification. Mol Cell Biol 25:1879–1890

    Article  CAS  PubMed  Google Scholar 

  • Lee K, Pisarska MD, Ko JJ, Kang Y, Yoon S, Ryou SM, Cha KY, Bae J (2005b) Transcriptional factor FOXL2 interacts with DP103 and induces apoptosis. Biochem Biophys Res Commun 336:876–881

    Article  CAS  PubMed  Google Scholar 

  • Lefebvre S, Bürglen L, Reboullet S, Clermont O, Burlet P, Viollet L, Benichou B, Cruaud C, Millasseau P, Zeviani M, Le Paslier D, Frezal J, Cohen D, Weissenbach J, Munnich A, Melki J (1995) Identification and characterization of a spinal muscular atrophy-determining gene. Cell 80:155–165

    Article  CAS  PubMed  Google Scholar 

  • Liu C, Gilmont RR, Benndorf R, Welsh MJ (2000) Identification and characterization of a novel protein from Sertoli cells, PASS1, that associates with mammalian small stress protein hsp27. J Biol Chem 275:18724–18731

    Article  CAS  PubMed  Google Scholar 

  • Marin-Vinader L, Shin C, Onnekink C, Manley JL, Lubsen NH (2006) Hsp27 enhances recovery of splicing as well as rephosphorylation of SRp38 after heat shock. Mol Biol Cell 17:886–894

    Article  CAS  PubMed  Google Scholar 

  • McWhorter ML, Monani UR, Burghes AH, Beattie CE (2003) Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding. J Cell Biol 162:919–931

    Article  CAS  PubMed  Google Scholar 

  • Mouillet JF, Yan X, Ou Q, Jin L, Muglia LJ, Crawford PA, Sadovsky Y (2008) DEAD-box protein-103 (DP103, Ddx20) is essential for early embryonic development and modulates ovarian morphology and function. Endocrinology 149:2168–21675

    Article  CAS  PubMed  Google Scholar 

  • Mymrikov EV, Bukach OV, Seit-Nebi AS, Gusev NB (2010)The pivotal role of the beta7 strand in the intersubunit contacts of different human small heat shock proteins. Cell Stress Chaperones, (in press)

  • Nelson PT, Hatzigeorgiou AG, Mourelatos Z (2004) miRNP:mRNA association in polyribosomes in a human neuronal cell line. RNA 10:387–394

    Article  CAS  PubMed  Google Scholar 

  • Ou Q, Mouillet JF, Yan X, Dorn C, Crawford PA, Sadovsky Y (2001) The DEAD box protein DP103 is a regulator of steroidogenic factor-1. Mol Endocrinol 15:69–79

    Article  CAS  PubMed  Google Scholar 

  • Pagliardini S, Giavazzi A, Setola V, Lizier C, Di Luca M, DeBiasi S, Battaglia G (2000) Subcellular localization and axonal transport of the survival motor neuron (SMN) protein in the developing rat spinal cord. Hum Mol Genet 9:47–56

    Article  CAS  PubMed  Google Scholar 

  • Pellizzoni L, Baccon J, Rappsilber J, Mann M, Dreyfuss G (2002) Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component. J Biol Chem 277:7540–7545

    Article  CAS  PubMed  Google Scholar 

  • Pilotto A, Marziliano N, Pasotti M, Grasso M, Costante AM, Arbustini E (2006) αB-crystallin mutation in dilated cardiomyopathies: low prevalence in a consecutive series of 200 unrelated probands. Biochem Biophys Res Commun 346:1115–1157

    Article  CAS  PubMed  Google Scholar 

  • Prior TW (2007) Spinal muscular atrophy diagnostics. J Child Neurol 22:952–956

    Article  PubMed  Google Scholar 

  • Roelofs MF, Boelens WC, Joosten LA, Abdollahi-Roodsaz S, Geurts J, Wunderink LU, Schreurs BW, van den Berg WB, Radstake TR (2006) Identification of small heat shock protein B8 (HSP22) as a novel TLR4 ligand and potential involvement in the pathogenesis of rheumatoid arthritis. J Immunol 176:7021–7027

    CAS  PubMed  Google Scholar 

  • Rogalla T, Ehrnsperger M, Préville X, Kotlyarov A, Lutsch G, Ducasse C, Paul C, Wieske M, Arrigo A-P, Buchner J, Gaestel M (1999) Regulation of Hsp27 oligomerization, chaperone function, and protective activity against oxidative stress/tumor necrosis factor α by phosphorylation. J Biol Chem 274:18947–18956

    Article  CAS  PubMed  Google Scholar 

  • Selcen D, Engel AG (2003) Myofibrillar myopathy caused by novel dominant negative αB-crystallin mutations. Ann Neurol 54:804–810

    Article  CAS  PubMed  Google Scholar 

  • Sharma A, Lambrechts A, le Hao T, Le TT, Sewry CA, Ampe C, Burghes AH, Morris GE (2005) A role for complexes of survival of motor neurons (SMN) protein with gemins and profilin in neurite-like cytoplasmic extensions of cultured nerve cells. Exp Cell Res 309:185–197

    Article  CAS  PubMed  Google Scholar 

  • Shchores K, Yehiely F, Kular RK, Kotlo KU, Brewer G, Deiss LP (2002) Cell death inhibiting RNA (CDIR) derived from a 3′-untranslated region binds AUF1 and heat shock protein 27. J Biol Chem 277:47061–47072

    Article  Google Scholar 

  • Shpargel KB, Matera AG (2005) Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins. Proc Natl Acad Sci USA 102:17372–17377

    Article  CAS  PubMed  Google Scholar 

  • Shy ME (2004) Charcot-Marie-Tooth disease: an update. Curr Opin Neurol 17:579–585

    Article  CAS  PubMed  Google Scholar 

  • Simon S, Fontaine JM, Martin JL, Sun X, Hoppe AD, Welsh MJ, Benndorf R, Vicart P (2007) Myopathy-associated alphaB-crystallin mutants: abnormal phosphorylation, intracellular location, and interactions with other small heat shock proteins. J Biol Chem 282:34276–34287

    Article  CAS  PubMed  Google Scholar 

  • Sinsimer KS, Gratacós FM, Knapinska AM, Lu J, Krause CD, Wierzbowski AV, Maher LR, Scrudato S, Rivera YM, Gupta S, Turrin DK, De La Cruz MP, Pestka S, Brewer G (2008) Chaperone Hsp27, a novel subunit of AUF1 protein complexes, functions in AU-rich element-mediated mRNA decay. Mol Cell Biol 28:5223–5237

    Article  CAS  PubMed  Google Scholar 

  • Sommer S, Cui Y, Brewer G, Fuqua SA (2005) The c-Yes 3′-UTR contains adenine/uridine-rich elements that bind AUF1 and HuR involved in mRNA decay in breast cancer cells. J Steroid Biochem Mol Biol 97:219–229

    Article  CAS  PubMed  Google Scholar 

  • Sun X, Fontaine J-M, Rest JS, Shelden EA, Welsh MJ, Benndorf R (2004) Interaction of human HSP22 (HSPB8) with other small heat shock proteins. J Biol Chem 279:2394–2402

    Article  CAS  PubMed  Google Scholar 

  • Sun X, Fontaine J-M, Bartl I, Behnam B, Welsh MJ, Benndorf R (2007) Induction of Hsp22 (HspB8) by estrogen and the metalloestrogen cadmium in estrogen receptor-positive breast cancer cells. Cell Stress Chaperones 12:307–319

    Article  CAS  PubMed  Google Scholar 

  • Tang BS, Zhao GH, Luo W, Xia K, Cai F, Pan Q, Zhang RX, Zhang FF, Liu XM, Chen B, Zhang C, Shen L, Jiang H, Long ZG, Dai HP (2005) Small heat-shock protein 22 mutated in autosomal dominant Charcot-Marie-Tooth disease type 2 L. Hum Genet 116:222–224

    Article  CAS  PubMed  Google Scholar 

  • Verschuure P, Tatard C, Boelens WC, Grongnet JF, David JC (2003) Expression of small heat shock proteins HspB2, HspB8, Hsp20 and cvHsp in different tissues of the perinatal developing pig. Eur J Cell Biol 82:523–530

    Article  CAS  PubMed  Google Scholar 

  • Vicart P, Caron A, Guicheney P, Li Z, Prévost MC, Faure A, Chateau D, Chapon F, Tome F, Dupret JM, Paulin D, Fardeau M (1998) A missense mutation in the αB-crystallin chaperone gene causes a desmin-related myopathy. Nat Genet 20:92–95

    Article  CAS  PubMed  Google Scholar 

  • Vos MJ, Kanon B, Kampinga HH (2009) HSPB7 is a SC35 speckle resident small heat shock protein. Biochim Biophys Acta 1793:1343–1353

    Article  CAS  PubMed  Google Scholar 

  • Wan L, Battle DJ, Yong J, Gubitz AK, Kolb SJ, Wang J, Dreyfuss G (2005) The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy. Mol Cell Biol 25:5543–5551

    Article  CAS  PubMed  Google Scholar 

  • Williams KL, Rahimtula M, Mearow KM (2006) Heat shock protein 27 is involved in neurite extension and branching of dorsal root ganglion neurons in vitro. J Neurosci Res 84:716–723

    Article  CAS  PubMed  Google Scholar 

  • Yan X, Mouillet JF, Ou Q, Sadovsky Y (2003) A novel domain within the DEAD-box protein DP103 is essential for transcriptional repression and helicase activity. Mol Cell Biol 23:414–423

    Article  CAS  PubMed  Google Scholar 

  • Zhang HL, Pan F, Hong D, Shenoy SM, Singer RH, Bassell GJ (2003) Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization. J Neurosci 23:6627–6637

    CAS  PubMed  Google Scholar 

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Acknowledgments

We thank L. Zhang (UM Center for Statistical Consultation and Research) for assistance with the statistical analysis of the qFRET data. The critical reading of the manuscript by Dr. D. Chandler (Columbus, OH) is gratefully acknowledged. The work was supported by Public Health Service grant P01ES11188 from the National Institute of Environmental Health Sciences to M.J.W. (PI) and R.B.; by a Munn Idea grant from the University of Michigan Comprehensive Cancer Center to R.B.; by Canadian Institutes of Health Research grant MOP-7088 to J.L.; by the French Ministry of Research and by the Association française contre les myopathies (AFM) to S.S.; and by the Centre national de la recherche scientifique (CNRS) and by the AFM grant No. 11764 to P.V.

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  1. Serena Carra

    Present address: Section for Radiation and Stress Cell Biology, Department of Cell Biology, University Medical Center Groningen, 9713 AV, Groningen, The Netherlands

Authors and Affiliations

  1. Department of Cell and Developmental Biology, University of Michigan Medical School, Ann Arbor, MI, 48109, USA

    Xiankui Sun, Jean-Marc Fontaine, Cheryl DeGuzman, Michael J. Welsh & Rainer Benndorf

  2. Department of Microbiology and Immunology, University of Michigan Medical School, Ann Arbor, MI, 48109, USA

    Adam D. Hoppe

  3. Le Centre de recherche en cancérologie, l’Université Laval, L’Hôtel-Dieu de Québec, Laval, Québec, Canada, G1R 2J6

    Serena Carra & Jacques Landry

  4. Department of Medicine, Cardiovascular Institute, Loyola University Medical Center, Maywood, IL, 60153, USA

    Jody L. Martin

  5. Laboratory BFA, University Paris Diderot/CNRS, 75013, Paris, France

    Stephanie Simon & Patrick Vicart

  6. Department of Pediatrics, Ohio State University, Columbus, OH, 43205, USA

    Rainer Benndorf

  7. The Center for Clinical and Translational Research, The Research Institute at Nationwide Children’s Hospital, Research Building II, Room WA2109, 700 Children’s Drive, Columbus, OH, 43205, USA

    Rainer Benndorf

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  1. Xiankui Sun
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Correspondence to Rainer Benndorf.

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Sun, X., Fontaine, JM., Hoppe, A.D. et al. Abnormal interaction of motor neuropathy-associated mutant HspB8 (Hsp22) forms with the RNA helicase Ddx20 (gemin3). Cell Stress and Chaperones 15, 567–582 (2010). https://doi.org/10.1007/s12192-010-0169-y

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