Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?

被引：147

作者：

Davies, SW

Beardsall, K

Turmaine, M

DiFiglia, M

Aronin, N

Bates, GP

机构：

[1] UCL, Dept Anat & Dev Biol, London WC1E 6JJ, England

[2] Harvard Univ, Massachusetts Gen Hosp, Sch Med, Dept Neurol, Boston, MA USA

[3] Univ Med & Dent New Jersey, Guys Hosp, Div Med & Mol Genet, London, England

来源：

LANCET | 1998年 / 351卷 / 9096期

关键词：

D O I：

10.1016/S0140-6736(97)08360-8

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Neuronal intranuclear inclusions have been found in the brain of a transgenic mouse model of Huntington's disease and in necropsy brain tissue of patients with Huntington's disease, We suggest that neuronal intranuclear inclusions are the common neuropathology for all inherited diseases caused by expansion of polyglutamine repeats, We also suggest that patients with a pathological diagnosis of neuronal intranuclear hyaline inclusion disease may also have polyglutamine repeat expansions.

引用

收藏

页码：131 / 133

页数：3

相关论文

共 39 条

[1]

[Anonymous], [No title captured]

[2] IDENTIFICATION AND CHARACTERIZATION OF THE GENE CAUSING TYPE-1 SPINOCEREBELLAR ATAXIA [J].

BANFI, S ;

SERVADIO, A ;

CHUNG, MY ;

KWIATKOWSKI, TJ ;

MCCALL, AE ;

DUVICK, LA ;

SHEN, Y ;

ROTH, EJ ;

ORR, HT ;

ZOGHBI, HY .

NATURE GENETICS, 1994, 7 (04) :513-520

[3] Transgenic models of Huntington's disease [J].

Bates, GP ;

Mangiarini, L ;

Mahal, A ;

Davies, SW .

HUMAN MOLECULAR GENETICS, 1997, 6 (10) :1633-1637

[4] Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation [J].

Davies, SW ;

Turmaine, M ;

Cozens, BA ;

DiFiglia, M ;

Sharp, AH ;

Ross, CA ;

Scherzinger, E ;

Wanker, EE ;

Mangiarini, L ;

Bates, GP .

CELL, 1997, 90 (03) :537-548

[5] Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain [J].

DiFiglia, M ;

Sapp, E ;

Chase, KO ;

Davies, SW ;

Bates, GP ;

Vonsattel, JP ;

Aronin, N .

SCIENCE, 1997, 277 (5334) :1990-1993

[6]

GILLES D, 1997, NAT GENET, V17, P65

[7] alpha-synuclein - a link between Parkinson and Alzheimer diseases? [J].

Heintz, N ;

Zoghbi, H .

NATURE GENETICS, 1997, 16 (04) :325-327

[8] Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats [J].

Imbert, G ;

Saudou, F ;

Yvert, G ;

Devys, D ;

Trottier, Y ;

Garnier, JM ;

Weber, C ;

Mandel, JL ;

Cancel, G ;

Abbas, N ;

Durr, A ;

Didierjean, O ;

Stevanin, G ;

Agid, Y ;

Brice, A .

NATURE GENETICS, 1996, 14 (03) :285-291

[9] WIDESPREAD INTRANUCLEAR NEURONAL CORPUSCLES (MARINESCO BODIES) ASSOCIATED WITH A FAMILIAL SPINAL DEGENERATION WITH CRANIAL AND PERIPHERAL-NERVE INVOLVEMENT [J].

JANOTA, I .

NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 1979, 5 (04) :311-317

[10] CAG EXPANSIONS IN A NOVEL GENE FOR MACHADO-JOSEPH DISEASE AT CHROMOSOME 14Q32.1 [J].

KAWAGUCHI, Y ;

OKAMOTO, T ;

TANIWAKI, M ;

AIZAWA, M ;

INOUE, M ;

KATAYAMA, S ;

KAWAKAMI, H ;

NAKAMURA, S ;

NISHIMURA, M ;

AKIGUCHI, I ;

KIMURA, J ;

NARUMIYA, S ;

KAKIZUKA, A .

NATURE GENETICS, 1994, 8 (03) :221-228

← 1 2 3 4 →