Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0016719 (Friedreich's ataxia)
 2,098 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Genetic experiments in mice, which are indispensable for studying the molecular basis of neurological disorders, have certain limitations that include slow pace and high costs. It is therefore not surprising that in recent years numerous neurological diseases have been modeled in genetically tractable organisms, including Drosophila, Caenorhabditis elegans, and yeast. Yeast models in particular have a special advantage with respect to genome-wide experimental approaches as a result of the completed sequencing of the genome, the availability of a collection of precise deletion mutants of every gene in the genome, and the rapidly evolving databases of yeast protein-protein interactions and gene expression patterns. These large and easily accessible bodies of information, coupled with the ease with which yeast can be manipulated genetically, have led to dissection of novel mechanisms of neurodegenerative disorders. In this review, we discuss how studies in yeast models have already resulted in significant insights into the understanding of neurodegenerative disorders that include prion disease, Parkinson's disease, polyglutamine expansion disorders, Friedreich's ataxia, and Batten disease.
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PMID:Making yeast tremble: yeast models as tools to study neurodegenerative disorders. 1452 57

In this chapter we are treating yeast cells as a model for oxidative stress response and the consequences of oxidative stress which are one cause for a number of human diseases, including neurodegenerative diseases, which form the main part of this paper. All such model building depends on orthologous relations between highly conserved yeast and human genes, which are easily recognized by sequence comparisons, but much more difficult to prove functionally. Previously we have treated Friedreich's ataxia, while presently we are describing in detail the neuronal ceroid lipofuscinoses, among them Batten disease. A general overview is given how yeast can aid current research in three of the most devastating and at the same time quantitatively most important neurodegenerative diseases of old age: Alzheimer's, Huntington's, and Parkinson's disease. In the ensuing part of the chapter, we describe yeast as model for metabolic regulation and hence as a model for inborn errors of metabolism that are in some instances very faithfully mirrored by introducing the same point mutations into yeast cells which are known from patients.
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PMID:Oxidative stress and neurodegeneration: the yeast model system. 2374 75