Animal models of human amyloidoses: Are transgenic mice worth the time and trouble?

被引：37

作者：

Buxbaum, Joel N. ^{[1
]}

机构：

[1] Scripps Res Inst, WM Keck Autoimmune Dis Ctr, Dept Mol & Expt Med, La Jolla, CA 92037 USA

来源：

FEBS LETTERS | 2009年 / 583卷 / 16期

基金：

美国国家卫生研究院;

关键词：

Amyloidosis; Transgenic model; Amyloid A; AL amyloid; Cystatin c; Gelsolin; Transthyretin; Alzheimer's disease; Serpinopathies; Familial dementias; APOLIPOPROTEIN-A-II; HUMAN TRANSTHYRETIN; ALZHEIMERS-DISEASE; PRECURSOR PROTEIN; BETA DEPOSITION; PARKINSONS-DISEASE; DIABETES-MELLITUS; ENHANCING FACTOR; MOUSE MODELS; MURINE MODEL;

D O I：

10.1016/j.febslet.2009.07.031

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：

The amyloidoses are the prototype gain of toxic function protein misfolding diseases. As such, several naturally occurring animal models and their inducible variants provided some of the first insights into these disorders of protein aggregation. With greater analytic knowledge and the increasing. exibility of transgenic and gene knockout technology, new models have been generated allowing the interrogation of phenomena that have not been approachable in more reductionist systems, i.e. behavioral readouts in the neurodegenerative diseases, interactions among organ systems in the transthyretin amyloidoses and taking pre-clinical therapeutic trials beyond cell culture. The current review describes the features of both transgenic and non-transgenic models and discusses issues that appear to be unresolved even when viewed in their organismal context. (C) 2009 Federation of European Biochemical Societies. Published by Elsevier B. V. All rights reserved.

引用

页码：2663 / 2673

页数：11

共 120 条

[1]

BAUMANN H, 1990, MOL BIOL MED, V7, P147

[2] Targeted suppression of an amyloidogenic transthyretin with antisense oligonucleotides [J].

Benson, MD ;

Kluve-Beckerman, B ;

Zeldenrust, SR ;

Siesky, AM ;

Bodenmiller, DM ;

Showalter, AD ;

Sloop, KW .

MUSCLE & NERVE, 2006, 33 (05) :609-618

[3] Induction of tau pathology by intracerebral infusion of amyloid-β-containing brain extract and by amyloid-β deposition in APP x tau transgenic mice [J].

Bolmont, Tristan ;

Clavaguera, Florence ;

Meyer-Luehmann, Melanie ;

Herzig, Martin C. ;

Radde, Rebecca ;

Staufenbiel, Matthias ;

Lewis, Jada ;

Hutton, Mike ;

Tolnay, Markus ;

Jucker, Mathias .

AMERICAN JOURNAL OF PATHOLOGY, 2007, 171 (06) :2012-2020

[4] Amyloid deposition is delayed in mice with targeted deletion of the serum amyloid P component gene [J].

Botto, M ;

Hawkins, PN ;

Bickerstaff, MCM ;

Herbert, J ;

Bygrave, AE ;

McBride, A ;

Hutchinson, WL ;

Tennent, GA ;

Walport, MJ ;

Pepys, MB .

NATURE MEDICINE, 1997, 3 (08) :855-859

[5] Tau alteration and neuronal degeneration in tauopathies: mechanisms and models [J].

Brandt, R ;

Hundelt, M ;

Shahani, N .

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, 2005, 1739 (2-3) :331-354

[6] Chemical chaperones mediate increased secretion of mutant α1-antitrypsin (α1-AT) Z:: A potential pharmacological strategy for prevention of liver injury and emphysema in α1-AT deficiency [J].

Burrows, JAJ ;

Willis, LK ;

Perlmutter, DH .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2000, 97 (04) :1796-1801

[7] The genetics of the amyloidoses: interactions with immunity and inflammation [J].

Buxbaum, J. .

GENES AND IMMUNITY, 2006, 7 (06) :439-449

[8] Transthyretin protects Alzheimer's mice from the behavioral and biochemical effects of Aβ toxicity [J].

Buxbaum, Joel N. ;

Ye, Zhengyi ;

Reixach, Natlia ;

Friske, Linsey ;

Levy, Coree ;

Das, Pritam ;

Golde, Todd ;

Masliah, Eliezer ;

Roberts, Amanda R. ;

Bartfai, Tamas .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2008, 105 (07) :2681-2686

[9] Genetic modification of the phenotypes produced by amyloid precursor protein overexpression in transgenic mice [J].

Carlson, GA ;

Borchelt, DR ;

Dake, A ;

Turner, S ;

Danielson, V ;

Coffin, JD ;

Eckman, C ;

Meiners, J ;

Nilsen, SP ;

Younkin, SG ;

Hsiao, KK .

HUMAN MOLECULAR GENETICS, 1997, 6 (11) :1951-1959

[10] Anti-amyloidogenic, anti-oxidant and anti-apoptotic role of gelsolin in Alzheimer's disease [J].

Chauhan, Ved ;

Ji, Lina ;

Chauhan, Abha .

BIOGERONTOLOGY, 2008, 9 (06) :381-389

← 1 2 3 4 5 6 7 8 9 10 →