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Familial Amyotrophic Lateral Sclerosis and Alzheimer’s Disease

Transgenic Models

  • Chapter
Molecular and Cellular Mechanisms of Neuronal Plasticity

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 446))

Abstract

Amyotrophic lateral sclerosis (ALS) and Alzheimer’s disease (AD) are two classical neurodegenerative disorders. These age-related, chronic, progressive diseases are accompanied by clinical signs that reflect the vulnerability and death of specific populations of neurons. Unfortunately, there are no satisfactory treatments for these diseases. Both ALS and AD may be inherited in an autosomal dominant fashion: some cases of familial ALS (FALS) are linked to mutations in Superoxide dismutase 1 (SOD1); and some individuals with familial AD (FAD) have mutations in genes encoding the amyloid precursor protein (APP) or presenilins (PS1 and PS2). Products of these mutant genes, thought to be associated with the formation of improperly folded or processed proteins, impact upon specific subsets of neural cells and cause characteristic clinical manifestations. For example, in ALS, damage to upper and lower motor neurons results in spasticity and weakness/muscle atrophy, respectively; in AD, the involvement of a variety of brain regions/neuronal populations is reflected in loss of memory, cognitive/behavioral impairments, and, eventually, profound dementia.

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Authors and Affiliations

  1. Department of Pathology, Baltimore, Maryland, USA

    Philip C. Wong, David R. Borchelt, Michael K. Lee, Carlos A. Pardo, Gopal Thinakaran, Lee J. Martin, Sangram S. Sisodia & Donald L. Price M.D.

  2. Neuropathology Laboratory, Baltimore, Maryland, USA

    Philip C. Wong, David R. Borchelt, Michael K. Lee, Carlos A. Pardo, Gopal Thinakaran, Lee J. Martin, Sangram S. Sisodia & Donald L. Price M.D.

  3. Department of Neuroscience, Baltimore, Maryland, USA

    Lee J. Martin, Sangram S. Sisodia & Donald L. Price M.D.

  4. Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

    Donald L. Price M.D.

  5. Neuropathology Laboratory, The Johns Hopkins University School of Medicine, 558 Ross Research Building 720 Rutland Avenue Baltimore, 21205-2196, Maryland, USA

    Donald L. Price M.D.

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  1. The College of Staten Island of The City University of New York, USA

    Yigal H. Ehrlich

  2. The CSI/IBR Center for Developmental Neuroscience Staten Island, New York, USA

    Yigal H. Ehrlich

  3. The City University Graduate School, New York, New York, USA

    Yigal H. Ehrlich

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Wong, P.C. et al. (1998). Familial Amyotrophic Lateral Sclerosis and Alzheimer’s Disease. In: Ehrlich, Y.H. (eds) Molecular and Cellular Mechanisms of Neuronal Plasticity. Advances in Experimental Medicine and Biology, vol 446. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-4869-0_9

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