Summary: Molecular Basis of the Neurodegenerative Disorders.
Neurodegenerative disorders are among the most devastating illnesses in western society. The disorders are chronic and progressive. Although these diseases are relatively common and often highly debilitating, the mechanisms responsible for their pathologies are poorly understood, and there are currently no effective preventative therapies. Over the past decade, many genes underlying heritable forms of the neurodegenerative disorders have been identified, including Alzheimer's disease, Parkinson's disease, Huntington's Disease and Friedreich's ataxia. Genetic anormalies that cause the neurodegenerative disorders are varied and complex. The disorders are characterized by the selective and symmetric loss of neurons in sensory, motor and cognitive systems. The challenge is to understand the pathogeneis of the neurogenerative disorders. There must be an understanding of the abnormalities in proteins transport, interaction and aggregation,.
The outline of the patterns of cell loss and the identification of disease specific cell markers help in nosologic classification. Alzheimer's disease is marked by senile plaques, neurofibrillary tangles, neuronal loss and the insufficient loss of the neurotransmitter acetylcholine. The depletion of dopamine is found in Parkinson's disease, as well as Lewy bodies, tiny deposits of protein placed in dying nerve cells, are found in damaged neurons deep within the brain. In Huntington's disease, cellular inclusions and swollen motor axions are found in amyotrophic lateral sclerosis. Also, y- aminobutyric acid that contains neurons of the neostraiatum are lost.
Many of the disorders have demonstrated Mendelian inheritance. Family inheritance plays an important part in the cases, such as in Huntington's disease. Autosomal dominant traits also are considered in diseases such as Alzheimer's disease and Parkinson's disease.