Transcriptional dysregulation in Huntington's disease

被引：324

作者：

Cha, JHJ ^{[1
]}

机构：

[1] Massachusetts Gen Hosp, Dept Neurol, Boston, MA 02114 USA

来源：

TRENDS IN NEUROSCIENCES | 2000年 / 23卷 / 09期

关键词：

D O I：

10.1016/S0166-2236(00)01609-X

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

Although the gene responsible for Huntington's disease was discovered in 1993, the pathogenic mechanisms by which mutant huntingtin causes neuronal dysfunction and death remain unclear. However, increasing evidence suggests that mutant huntingtin disrupts the normal transcriptional program of susceptible neurons. Thus, transcriptional dysregulation might be an important pathogenic mechanism in Huntington's disease.

引用

页码：387 / 392

页数：6

共 103 条

[31] D1 AND D2 DOPAMINE RECEPTOR REGULATED GENE-EXPRESSION OF STRIATONIGRAL AND STRIATOPALLIDAL NEURONS [J].

GERFEN, CR ;

ENGBER, TM ;

MAHAN, LC ;

SUSEL, Z ;

CHASE, TN ;

MONSMA, FJ ;

SIBLEY, DR .

SCIENCE, 1990, 250 (4986) :1429-1432

[32] Huntingtin: a single bait hooks many species [J].

Gusella, JF ;

MacDonald, ME .

CURRENT OPINION IN NEUROBIOLOGY, 1998, 8 (03) :425-430

[33] The genetic defect causing Huntington's disease: Repeated in other contexts? [J].

Gusella, JF ;

Persichetti, F ;

MacDonald, ME .

MOLECULAR MEDICINE, 1997, 3 (04) :238-246

[34] In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease [J].

Hackam, AS ;

Singaraja, R ;

Zhang, TQ ;

Gan, L ;

Hayden, MR .

HUMAN MOLECULAR GENETICS, 1999, 8 (01) :25-33

[35] The influence of Huntingtin protein size on nuclear localization and cellular toxicity [J].

Hackam, AS ;

Singaraja, R ;

Wellington, CL ;

Metzler, M ;

McCutcheon, K ;

Zhang, TQ ;

Kalchman, M ;

Hayden, MR .

JOURNAL OF CELL BIOLOGY, 1998, 141 (05) :1097-1105

[36] UBIQUITIN, PROTEASOMES, AND THE REGULATION OF INTRACELLULAR PROTEIN-DEGRADATION [J].

HOCHSTRASSER, M .

CURRENT OPINION IN CELL BIOLOGY, 1995, 7 (02) :215-223

[37] A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration [J].

Hodgson, JG ;

Agopyan, N ;

Gutekunst, CA ;

Leavitt, BR ;

LePiane, F ;

Singaraja, R ;

Smith, DJ ;

Bissada, N ;

McCutcheon, K ;

Nasir, J ;

Jamot, L ;

Li, XJ ;

Stevens, ME ;

Rosemond, E ;

Roder, JC ;

Phillips, AG ;

Rubin, EM ;

Hersch, SM ;

Hayden, MR .

NEURON, 1999, 23 (01) :181-192

[38] CHARACTERIZATION AND LOCALIZATION OF THE HUNTINGTON DISEASE GENE-PRODUCT [J].

HOOGEVEEN, AT ;

WILLEMSEN, R ;

MEYER, N ;

DEROOIJ, KE ;

ROOS, RAC ;

VANOMMEN, GJB ;

GALJAARD, H .

HUMAN MOLECULAR GENETICS, 1993, 2 (12) :2069-2073

[39] Amyloid formation by mutant huntingtin: Threshold, progressivity and recruitment of normal polyglutamine proteins [J].

Huang, CC ;

Faber, PW ;

Persichetti, F ;

Mittal, V ;

Vonsattel, JP ;

MacDonald, ME ;

Gusella, JF .

SOMATIC CELL AND MOLECULAR GENETICS, 1998, 24 (04) :217-233

[40] Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons [J].

Jackson, GR ;

Salecker, I ;

Dong, XZ ;

Yao, X ;

Arnheim, N ;

Faber, PW ;

MacDonald, ME ;

Zipursky, SL .

NEURON, 1998, 21 (03) :633-642

← 1 2 3 4 5 6 7 8 9 10 →