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Transgenic Mouse Models and Human Neurodegenerative Disorders
Han-Xiang Deng, MD, PhD;
Teepu Siddique, MD
Arch Neurol. 2000;57:1695-1702.
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INTRODUCTION
The development of new methods for manipulating the mouse genome by transgenic and gene-targeting technologies has dramatically increased our ability to create mouse models for human genetic diseases. These mouse models have greatly facilitated the understanding of the pathogenesis of some human diseases and are beginning to be used in screening of therapeutic agents. In this review, we outline 2 basic techniques that are most frequently used to alter the mouse genetic makeup and summarize their application in the study of some common neurodegenerative disorders.
BASIC TECHNOLOGIES
There are 2 basic technologies to generate mouse models: transgenesis and gene targeting.1-3 In general, when the disease trait is dominant, ie, either a gain-of-function or a dominant-negative effect mutation leads to the disease, the mouse model can be established through transgenic methods. When the disease is recessive, ie, a loss-of-function mutation causes the disease, the mouse model . . . [Full Text of this Article]
TRANSGENESIS
Microinjection of DNA Into the Pronucleus Retrovirus Infection Embryonic Stem Cells Basic Procedures
GENE TARGETING
MOUSE MODELS FOR NEURODEGENERATIVE DISORDERS
AMYOTROPHIC LATERAL SCLEROSIS
Apoptosis Inclusions Neurofilaments
GLUTAMINE EXPANSION DISORDERS
ALZHEIMER DISEASE
Amyloid Precursor Protein PS1 and PS2 Apolipoprotein E
PRION DISEASES
CONCLUSIONS
From the Department of Neurology, Northwestern University Medical School, Chicago, Ill.
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ABSTRACT
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