Genetic neurodegenerative diseases: The human illness and transgenic models

被引：216

作者：

Price, DL

Sisodia, SS

Borchelt, DR

机构：

[1] Johns Hopkins Univ, Sch Med, Div Neuropathol, Dept Pathol, Baltimore, MD 21205 USA

[2] Johns Hopkins Univ, Sch Med, Dept Neurol, Baltimore, MD 21205 USA

[3] Johns Hopkins Univ, Sch Med, Dept Neurosci, Baltimore, MD 21205 USA

[4] Johns Hopkins Univ, Sch Med, Neuropathol Lab, Baltimore, MD 21205 USA

[5] Univ Chicago, Dept Pharmacol & Physiol Sci, Chicago, IL 60637 USA

来源：

SCIENCE | 1998年 / 282卷 / 5391期

关键词：

D O I：

10.1126/science.282.5391.1079

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

The neurodegenerative disorders, a heterogeneous group of chronic progressive diseases, are among the most puzzling and devastating illnesses in medicine. Some of these disorders, such as Alzheimer's disease, amyotrophic lateral sclerosis, the prion diseases, and Parkinson's disease, can occur sporadically and, in some instances, are caused by inheritance of gene mutations. Huntington's disease is acquired in an entirely genetic manner. Transgenic mice that express disease-causing genes recapitulate many features of these diseases. This review provides an overview of transgenic mouse models of familiar amyotrophic lateral sclerosis, familiar Alzheimer's disease, and Huntington's disease and the emerging insights relevant to the underlying molecular mechanisms of these diseases.

引用

收藏

页码：1079 / 1083

页数：5

相关论文

共 112 条

[51] VISUALIZATION OF A-BETA-42(43) AND A-BETA-40 IN SENILE PLAQUES WITH END-SPECIFIC A-BETA MONOCLONALS - EVIDENCE THAT AN INITIALLY DEPOSITED SPECIES IS A-BETA-42(43) [J].

IWATSUBO, T ;

ODAKA, A ;

SUZUKI, N ;

MIZUSAWA, H ;

NUKINA, N ;

IHARA, Y .

NEURON, 1994, 13 (01) :45-53

[52] SEEDING ONE-DIMENSIONAL CRYSTALLIZATION OF AMYLOID - A PATHOGENIC MECHANISM IN ALZHEIMERS-DISEASE AND SCRAPIE [J].

JARRETT, JT ;

LANSBURY, PT .

CELL, 1993, 73 (06) :1055-1058

[53] Machado-Joseph disease and SCA3: The genotype meets the phenotypes [J].

Junck, L ;

Fink, JK .

NEUROLOGY, 1996, 46 (01) :4-8

[54] DEPOSITION OF BETA/A4 IMMUNOREACTIVITY AND NEURONAL PATHOLOGY IN TRANSGENIC MICE EXPRESSING THE CARBOXYL-TERMINAL FRAGMENT OF THE ALZHEIMER AMYLOID PRECURSOR IN THE BRAIN [J].

KAMMESHEIDT, A ;

BOYCE, FM ;

SPANOYANNIS, AF ;

CUMMINGS, BJ ;

ORTEGON, M ;

COTMAN, C ;

VAUGHT, JL ;

NEVE, RL .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1992, 89 (22) :10857-10861

[55] THE PRECURSOR OF ALZHEIMERS-DISEASE AMYLOID-A4 PROTEIN RESEMBLES A CELL-SURFACE RECEPTOR [J].

KANG, J ;

LEMAIRE, HG ;

UNTERBECK, A ;

SALBAUM, JM ;

MASTERS, CL ;

GRZESCHIK, KH ;

MULTHAUP, G ;

BEYREUTHER, K ;

MULLERHILL, B .

NATURE, 1987, 325 (6106) :733-736

[56] The CAG/polyglutamine tract diseases: Gene products and molecular pathogenesis [J].

Koshy, BT ;

Zoghbi, HY .

BRAIN PATHOLOGY, 1997, 7 (03) :927-942

[57] Bcl-2: Prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis [J].

Kostic, V ;

JacksonLewis, V ;

deBilbao, F ;

DuboisDauphin, M ;

Przedborski, S .

SCIENCE, 1997, 277 (5325) :559-562

[58] THE ALZHEIMERS A-BETA PEPTIDE INDUCES NEURODEGENERATION AND APOPTOTIC CELL-DEATH IN TRANSGENIC MICE [J].

LAFERLA, FM ;

TINKLE, BT ;

BIEBERICH, CJ ;

HAUDENSCHILD, CC ;

JAY, G .

NATURE GENETICS, 1995, 9 (01) :21-30

[59] INTRODUCTION AND EXPRESSION OF THE 400 KILOBASE PRECURSOR AMYLOID PROTEIN GENE IN TRANSGENIC MICE [J].

LAMB, BT ;

SISODIA, SS ;

LAWLER, AM ;

SLUNT, HH ;

KITT, CA ;

KEARNS, WG ;

PEARSON, PL ;

PRICE, DL ;

GEARHART, JD .

NATURE GENETICS, 1993, 5 (01) :22-29

[60] Altered metabolism of familial Alzheimer's disease-linked amyloid precursor protein variants in yeast artificial chromosome transgenic mice [J].

Lamb, BT ;

Call, LM ;

Slunt, HH ;

Bardel, KA ;

Lawler, AM ;

Eckman, CB ;

Younkin, SG ;

Holtz, G ;

Wagner, SL ;

Price, DL ;

Sisodia, SS ;

Gearhart, JD .

HUMAN MOLECULAR GENETICS, 1997, 6 (09) :1535-1541

← 1 2 3 4 5 6 7 8 9 10 →