메뉴 건너뛰기




Volumn 167, Issue 3, 2016, Pages 774-788.e17

C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

Author keywords

amyotrophic lateral sclerosis; C9ORF72; dipeptide repeat; membrane less organelle; nucleolus; phase separation; stress granule

Indexed keywords

ARGININE; ARGININE DERIVATIVE; BINDING PROTEIN; DIPEPTIDE; DIPEPTIDE REPEAT PROTEIN; POLYGLYCYLARGININE; POLYPROLYLARGININE; PROTEIN; RNA BINDING PROTEIN; UNCLASSIFIED DRUG; C9ORF72 PROTEIN, HUMAN; PEPTIDE; POLYARGININE; POLYGLYCINE; POLYPROLINE; REPETITIVE DNA;

EID: 84992371797     PISSN: 00928674     EISSN: 10974172     Source Type: Journal    
DOI: 10.1016/j.cell.2016.10.002     Document Type: Article
Times cited : (537)

References (60)
  • 2
    • 66249103703 scopus 로고    scopus 로고
    • RNA granules: post-transcriptional and epigenetic modulators of gene expression
    • Anderson, P., Kedersha, N., RNA granules: post-transcriptional and epigenetic modulators of gene expression. Nat. Rev. Mol. Cell Biol. 10 (2009), 430–436.
    • (2009) Nat. Rev. Mol. Cell Biol. , vol.10 , pp. 430-436
    • Anderson, P.1    Kedersha, N.2
  • 4
    • 79952723337 scopus 로고    scopus 로고
    • Active liquid-like behavior of nucleoli determines their size and shape in Xenopus laevis oocytes
    • Brangwynne, C.P., Mitchison, T.J., Hyman, A.A., Active liquid-like behavior of nucleoli determines their size and shape in Xenopus laevis oocytes. Proc. Natl. Acad. Sci. USA 108 (2011), 4334–4339.
    • (2011) Proc. Natl. Acad. Sci. USA , vol.108 , pp. 4334-4339
    • Brangwynne, C.P.1    Mitchison, T.J.2    Hyman, A.A.3
  • 5
    • 84946554664 scopus 로고    scopus 로고
    • Polymer physics of intracellular phase transitions
    • Brangwynne, C., Tompa, P., Pappu, R., Polymer physics of intracellular phase transitions. Nat. Phys. 11 (2015), 899–904.
    • (2015) Nat. Phys. , vol.11 , pp. 899-904
    • Brangwynne, C.1    Tompa, P.2    Pappu, R.3
  • 6
    • 84956943850 scopus 로고    scopus 로고
    • GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function
    • Burguete, A.S., Almeida, S., Gao, F.B., Kalb, R., Akins, M.R., Bonini, N.M., GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function. eLife, 4, 2015, e08881.
    • (2015) eLife , vol.4 , pp. e08881
    • Burguete, A.S.1    Almeida, S.2    Gao, F.B.3    Kalb, R.4    Akins, M.R.5    Bonini, N.M.6
  • 7
    • 84855425542 scopus 로고    scopus 로고
    • Fluorescent fusion protein knockout mediated by anti-GFP nanobody
    • Caussinus, E., Kanca, O., Affolter, M., Fluorescent fusion protein knockout mediated by anti-GFP nanobody. Nat. Struct. Mol. Biol. 19 (2011), 117–121.
    • (2011) Nat. Struct. Mol. Biol. , vol.19 , pp. 117-121
    • Caussinus, E.1    Kanca, O.2    Affolter, M.3
  • 10
    • 0000857494 scopus 로고
    • An approach to correlate tandem mass spectral data of peptides with amino acid sequences in a protein database
    • Eng, J.K., McCormack, A.L., Yates, J.R., An approach to correlate tandem mass spectral data of peptides with amino acid sequences in a protein database. J. Am. Soc. Mass Spectrom 5 (1994), 976–989.
    • (1994) J. Am. Soc. Mass Spectrom , vol.5 , pp. 976-989
    • Eng, J.K.1    McCormack, A.L.2    Yates, J.R.3
  • 17
    • 84913550995 scopus 로고    scopus 로고
    • Amino acid composition of proteins reduces deleterious impact of mutations
    • Hormoz, S., Amino acid composition of proteins reduces deleterious impact of mutations. Sci. Rep., 3, 2013, 2919.
    • (2013) Sci. Rep. , vol.3 , pp. 2919
    • Hormoz, S.1
  • 18
    • 61449172037 scopus 로고    scopus 로고
    • Systematic and integrative analysis of large gene lists using DAVID bioinformatics resources
    • Huang da, W., Sherman, B.T., Lempicki, R.A., Systematic and integrative analysis of large gene lists using DAVID bioinformatics resources. Nat Protoc 4 (2009), 44–57.
    • (2009) Nat Protoc , vol.4 , pp. 44-57
    • Huang da, W.1    Sherman, B.T.2    Lempicki, R.A.3
  • 21
    • 84860872161 scopus 로고    scopus 로고
    • Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogels
    • Kato, M., Han, T.W., Xie, S., Shi, K., Du, X., Wu, L.C., Mirzaei, H., Goldsmith, E.J., Longgood, J., Pei, J., et al. Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogels. Cell 149 (2012), 753–767.
    • (2012) Cell , vol.149 , pp. 753-767
    • Kato, M.1    Han, T.W.2    Xie, S.3    Shi, K.4    Du, X.5    Wu, L.C.6    Mirzaei, H.7    Goldsmith, E.J.8    Longgood, J.9    Pei, J.10
  • 25
    • 84940503517 scopus 로고    scopus 로고
    • Promiscuous interactions and protein disaggregases determine the material state of stress-inducible RNP granules
    • Kroschwald, S., Maharana, S., Mateju, D., Malinovska, L., Nüske, E., Poser, I., Richter, D., Alberti, S., Promiscuous interactions and protein disaggregases determine the material state of stress-inducible RNP granules. eLife, 4, 2015, e06807.
    • (2015) eLife , vol.4 , pp. e06807
    • Kroschwald, S.1    Maharana, S.2    Mateju, D.3    Malinovska, L.4    Nüske, E.5    Poser, I.6    Richter, D.7    Alberti, S.8
  • 27
    • 84907221451 scopus 로고    scopus 로고
    • Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells
    • Kwon, I., Xiang, S., Kato, M., Wu, L., Theodoropoulos, P., Wang, T., Kim, J., Yun, J., Xie, Y., McKnight, S.L., Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells. Science 345 (2014), 1139–1145.
    • (2014) Science , vol.345 , pp. 1139-1145
    • Kwon, I.1    Xiang, S.2    Kato, M.3    Wu, L.4    Theodoropoulos, P.5    Wang, T.6    Kim, J.7    Yun, J.8    Xie, Y.9    McKnight, S.L.10
  • 29
    • 84944884978 scopus 로고    scopus 로고
    • Formation and Maturation of Phase-Separated Liquid Droplets by RNA-Binding Proteins
    • Lin, Y., Protter, D.S., Rosen, M.K., Parker, R., Formation and Maturation of Phase-Separated Liquid Droplets by RNA-Binding Proteins. Mol. Cell 60 (2015), 208–219.
    • (2015) Mol. Cell , vol.60 , pp. 208-219
    • Lin, Y.1    Protter, D.S.2    Rosen, M.K.3    Parker, R.4
  • 30
    • 84992491195 scopus 로고    scopus 로고
    • Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers
    • this issue
    • Lin, Y., Mori, E., Kato, M., Xiang, S., Wu, L., Kwon, I., McKnight, S., Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers. Cell 167 (2016), 789–802 this issue.
    • (2016) Cell , vol.167 , pp. 789-802
    • Lin, Y.1    Mori, E.2    Kato, M.3    Xiang, S.4    Wu, L.5    Kwon, I.6    McKnight, S.7
  • 34
    • 84961279195 scopus 로고    scopus 로고
    • Nucleophosmin integrates within the nucleolus via multi-modal interactions with proteins displaying R-rich linear motifs and rRNA
    • Mitrea, D.M., Cika, J.A., Guy, C.S., Ban, D., Banerjee, P.R., Stanley, C.B., Nourse, A., Deniz, A.A., Kriwacki, R.W., Nucleophosmin integrates within the nucleolus via multi-modal interactions with proteins displaying R-rich linear motifs and rRNA. eLife, 5, 2016, e13571.
    • (2016) eLife , vol.5 , pp. e13571
    • Mitrea, D.M.1    Cika, J.A.2    Guy, C.S.3    Ban, D.4    Banerjee, P.R.5    Stanley, C.B.6    Nourse, A.7    Deniz, A.A.8    Kriwacki, R.W.9
  • 36
    • 84944907005 scopus 로고    scopus 로고
    • Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillization
    • Molliex, A., Temirov, J., Lee, J., Coughlin, M., Kanagaraj, A.P., Kim, H.J., Mittag, T., Taylor, J.P., Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillization. Cell 163 (2015), 123–133.
    • (2015) Cell , vol.163 , pp. 123-133
    • Molliex, A.1    Temirov, J.2    Lee, J.3    Coughlin, M.4    Kanagaraj, A.P.5    Kim, H.J.6    Mittag, T.7    Taylor, J.P.8
  • 38
    • 84960129723 scopus 로고    scopus 로고
    • ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function
    • Murakami, T., Qamar, S., Lin, J.Q., Schierle, G.S., Rees, E., Miyashita, A., Costa, A.R., Dodd, R.B., Chan, F.T., Michel, C.H., et al. ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function. Neuron 88 (2015), 678–690.
    • (2015) Neuron , vol.88 , pp. 678-690
    • Murakami, T.1    Qamar, S.2    Lin, J.Q.3    Schierle, G.S.4    Rees, E.5    Miyashita, A.6    Costa, A.R.7    Dodd, R.B.8    Chan, F.T.9    Michel, C.H.10
  • 39
    • 77957011660 scopus 로고    scopus 로고
    • Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy
    • Nedelsky, N.B., Pennuto, M., Smith, R.B., Palazzolo, I., Moore, J., Nie, Z., Neale, G., Taylor, J.P., Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy. Neuron 67 (2010), 936–952.
    • (2010) Neuron , vol.67 , pp. 936-952
    • Nedelsky, N.B.1    Pennuto, M.2    Smith, R.B.3    Palazzolo, I.4    Moore, J.5    Nie, Z.6    Neale, G.7    Taylor, J.P.8
  • 42
    • 84902160274 scopus 로고    scopus 로고
    • Intrinsically disordered proteins and intrinsically disordered protein regions
    • Oldfield, C.J., Dunker, A.K., Intrinsically disordered proteins and intrinsically disordered protein regions. Annu. Rev. Biochem. 83 (2014), 553–584.
    • (2014) Annu. Rev. Biochem. , vol.83 , pp. 553-584
    • Oldfield, C.J.1    Dunker, A.K.2
  • 47
    • 63949084607 scopus 로고    scopus 로고
    • SUnSET, a nonradioactive method to monitor protein synthesis
    • Schmidt, E.K., Clavarino, G., Ceppi, M., Pierre, P., SUnSET, a nonradioactive method to monitor protein synthesis. Nat. Methods 6 (2009), 275–277.
    • (2009) Nat. Methods , vol.6 , pp. 275-277
    • Schmidt, E.K.1    Clavarino, G.2    Ceppi, M.3    Pierre, P.4
  • 48
    • 85012041135 scopus 로고    scopus 로고
    • Decoding ALS from Genes to Mechanism
    • Published online November 10, 2016
    • Taylor, J.P., Brown, R.J., Cleveland, D.W., Decoding ALS from Genes to Mechanism. Nature, 2016, 10.1038/nature20413 Published online November 10, 2016.
    • (2016) Nature
    • Taylor, J.P.1    Brown, R.J.2    Cleveland, D.W.3
  • 49
    • 84946069451 scopus 로고    scopus 로고
    • UniProt: a hub for protein information
    • UniProt Consortium. UniProt: a hub for protein information. Nucleic Acids Res. 43 (2015), D204–D212.
    • (2015) Nucleic Acids Res. , vol.43 , pp. D204-D212
  • 52
    • 0027305616 scopus 로고
    • Fluorescent labeling of nascent RNA reveals transcription by RNA polymerase II in domains scattered throughout the nucleus
    • Wansink, D.G., Schul, W., van der Kraan, I., van Steensel, B., van Driel, R., de Jong, L., Fluorescent labeling of nascent RNA reveals transcription by RNA polymerase II in domains scattered throughout the nucleus. J. Cell Biol. 122 (1993), 283–293.
    • (1993) J. Cell Biol. , vol.122 , pp. 283-293
    • Wansink, D.G.1    Schul, W.2    van der Kraan, I.3    van Steensel, B.4    van Driel, R.5    de Jong, L.6
  • 53
    • 84926357619 scopus 로고    scopus 로고
    • Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death
    • Wen, X., Tan, W., Westergard, T., Krishnamurthy, K., Markandaiah, S.S., Shi, Y., Lin, S., Shneider, N.A., Monaghan, J., Pandey, U.B., et al. Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death. Neuron 84 (2014), 1213–1225.
    • (2014) Neuron , vol.84 , pp. 1213-1225
    • Wen, X.1    Tan, W.2    Westergard, T.3    Krishnamurthy, K.4    Markandaiah, S.S.5    Shi, Y.6    Lin, S.7    Shneider, N.A.8    Monaghan, J.9    Pandey, U.B.10
  • 54
    • 0029901640 scopus 로고    scopus 로고
    • Analysis of compositionally biased regions in sequence databases
    • Wootton, J.C., Federhen, S., Analysis of compositionally biased regions in sequence databases. Methods Enzymol. 266 (1996), 554–571.
    • (1996) Methods Enzymol. , vol.266 , pp. 554-571
    • Wootton, J.C.1    Federhen, S.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.