Huntington’s Disease and Amyotrophic Lateral Sclerosis

  • Martin Beckerman
Part of the Biological and Medical Physics, Biomedical Engineering book series (BIOMEDICAL)

Huntington’s disease is a fatal disorder affecting neurons in the brain resulting in motor dysfunction and progressing to cognitive decline, dementia, and death some 15–20 years after onset of the illness. This disease was first described by George Huntington in 1872. The primary targets are spiny neurons situated in the striatum that release the neurotransmitter γ-aminobutyric acid (GABA) but in later stages of the illness populations of neurons in the hippocampus and cortex are affected.

The causal agent of the disease is mutated forms of Huntingtin (Htt), a large, highly flexible, and most likely natively unfolded signaling protein composed of 3144 amino acid residues. The most prominent feature of this protein from the disease viewpoint is the presence of large tract of polyglutamine (PolyQ) repeats in the N-terminal region. In individuals with Huntington’s disease, there are too many of these repeats. As will be discussed shortly, in the first part of this chapter, the Htt protein...


Amyotrophic Lateral Sclerosis Motor Neuron Mutant SOD1 Huntingtin Protein Heat Repeat 
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Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  1. 1.Oak RidgeUSA

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