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Saccharomyces cerevisiae Models of Alzheimer's Disease to Screen Genes, Mutations, and Chemicals Affecting Amyloid Beta Production by γ-Secretase
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Elevation in sphingomyelin synthase activity is associated with increases in amyloid-beta peptide generation

Jen-Hsiang T Hsiao1, Yuhong Fu, Andrew F Hill

  • 1Neuroscience Research Australia, Sydney, New South Wales, Australia.

Plos One
|August 27, 2013
PubMed
Summary

Sphingomyelin synthase (SGMS1) activity impacts amyloid-beta (Aβ) production, a hallmark of Alzheimer's disease (AD). Inhibiting SGMS1 reduces Aβ levels, suggesting SGMS1 as a potential factor in AD pathology.

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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...