Transgenic animal models of neurodegeneration based on human genetic studies.

Abstract

The identification of genes linked to neurodegenerative diseases such as Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS), Huntington's disease (HD) and Parkinson's disease (PD) has led to the development of animal models for studying mechanism and evaluating potential therapies. None of the transgenic models developed based on disease-associated genes have been able to fully recapitulate the behavioral and pathological features of the corresponding disease. However, there has been enormous progress made in identifying potential therapeutic targets and understanding some of the common mechanisms of neurodegeneration. In this review, we will discuss transgenic animal models for AD, ALS, HD and PD that are based on human genetic studies. All of the diseases discussed have active or complete clinical trials for experimental treatments that benefited from transgenic models of the disease.

• Authors: Brandon K Harvey, Christopher T Richie, Barry J Hoffer, Mikko Airavaara
• Journal: Journal of neural transmission (Vienna, Austria : 1996) (J Neural Transm (Vienna)) Vol. 118 Issue 1 Pg. 27-45 (Jan 2011) ISSN: 1435-1463 [Electronic] Austria
• PMID: 20931247 (Publication Type: Journal Article, Research Support, N.I.H., Intramural, Review)

Topics

• Alzheimer Disease (genetics, pathology)
• Amyotrophic Lateral Sclerosis (genetics, pathology)
• Animals
• Animals, Genetically Modified
• Disease Models, Animal
• Humans
• Huntington Disease (genetics, pathology)
• Nerve Degeneration (genetics, pathology)
• Parkinson Disease (genetics, pathology)