Huntingtin fragments that aggregate go their separate ways

被引:19
作者
DiFiglia, M [1 ]
机构
[1] Massachusetts Gen Hosp, Dept Neurol, Charlestown, MA 02114 USA
关键词
D O I
10.1016/S1097-2765(02)00609-3
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
N-terminal region of mutant huntingtin forms intranuclear and cytoplasmic aggregates in neurons that may contribute to neuronal death in Huntington's disease. Lunkes et al. (2002) show that different endoprotease-cleaved huntingtin fragments form nuclear and cytoplasmic inclusions.
引用
收藏
页码:224 / 225
页数:2
相关论文
共 11 条
  • [1] Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
    Davies, SW
    Turmaine, M
    Cozens, BA
    DiFiglia, M
    Sharp, AH
    Ross, CA
    Scherzinger, E
    Wanker, EE
    Mangiarini, L
    Bates, GP
    [J]. CELL, 1997, 90 (03) : 537 - 548
  • [2] Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
    DiFiglia, M
    Sapp, E
    Chase, KO
    Davies, SW
    Bates, GP
    Vonsattel, JP
    Aronin, N
    [J]. SCIENCE, 1997, 277 (5334) : 1990 - 1993
  • [3] Mutant protein in Huntington disease is resistant to proteolysis in affected brain
    Dyer, RB
    McMurray, CT
    [J]. NATURE GENETICS, 2001, 29 (03) : 270 - 278
  • [4] Calpain activation in Huntington's disease
    Gafni, J
    Ellerby, LM
    [J]. JOURNAL OF NEUROSCIENCE, 2002, 22 (12) : 4842 - 4849
  • [5] Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis
    Kim, YJ
    Yi, Y
    Sapp, E
    Wang, YM
    Cuiffo, B
    Kegel, KB
    Qin, ZH
    Aronin, N
    DiFiglia, M
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2001, 98 (22) : 12784 - 12789
  • [6] Proteases acting on mutant Huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions
    Lunkes, A
    Lindenberg, KS
    Ben-Haïem, L
    Weber, C
    Devys, D
    Landwehrmeyer, GB
    Mandel, JL
    Trottier, Y
    [J]. MOLECULAR CELL, 2002, 10 (02) : 259 - 269
  • [7] A NOVEL GENE CONTAINING A TRINUCLEOTIDE REPEAT THAT IS EXPANDED AND UNSTABLE ON HUNTINGTONS-DISEASE CHROMOSOMES
    MACDONALD, ME
    AMBROSE, CM
    DUYAO, MP
    MYERS, RH
    LIN, C
    SRINIDHI, L
    BARNES, G
    TAYLOR, SA
    JAMES, M
    GROOT, N
    MACFARLANE, H
    JENKINS, B
    ANDERSON, MA
    WEXLER, NS
    GUSELLA, JF
    BATES, GP
    BAXENDALE, S
    HUMMERICH, H
    KIRBY, S
    NORTH, M
    YOUNGMAN, S
    MOTT, R
    ZEHETNER, G
    SEDLACEK, Z
    POUSTKA, A
    FRISCHAUF, AM
    LEHRACH, H
    BUCKLER, AJ
    CHURCH, D
    DOUCETTESTAMM, L
    ODONOVAN, MC
    RIBARAMIREZ, L
    SHAH, M
    STANTON, VP
    STROBEL, SA
    DRATHS, KM
    WALES, JL
    DERVAN, P
    HOUSMAN, DE
    ALTHERR, M
    SHIANG, R
    THOMPSON, L
    FIELDER, T
    WASMUTH, JJ
    TAGLE, D
    VALDES, J
    ELMER, L
    ALLARD, M
    CASTILLA, L
    SWAROOP, M
    [J]. CELL, 1993, 72 (06) : 971 - 983
  • [8] Glutamine repeats and neurodegenerative diseases: molecular aspects
    Perutz, MF
    [J]. TRENDS IN BIOCHEMICAL SCIENCES, 1999, 24 (02) : 58 - 63
  • [9] Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
    Scherzinger, E
    Lurz, R
    Turmaine, M
    Mangiarini, L
    Hollenbach, B
    Hasenbank, R
    Bates, GP
    Davies, SW
    Lehrach, H
    Wanker, EE
    [J]. CELL, 1997, 90 (03) : 549 - 558
  • [10] Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA
    Schilling, G
    Wood, JD
    Kui, DA
    Slunt, HH
    Gonzales, V
    Yamada, M
    Cooper, JK
    Margolis, RL
    Jenkins, NA
    Copeland, NG
    Takahashi, H
    Tsuji, S
    Price, DL
    Borchelt, DR
    Ross, CA
    [J]. NEURON, 1999, 24 (01) : 275 - 286