Pathological and functional amyloid formation orchestrated by the secretory pathway

Academic Article
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related to degree

  • Pelletier, Mary Elizabeth Huff, Ph.D. in Chemistry, Scripps Research 1998 - 2003

publication date

  • 2003

abstract

  • Amyloidogenesis has historically been associated with pathology in a class of neurodegenerative diseases known as amyloid diseases. Recent studies have shown that proteolysis by furin during secretion initiates both variant gelsolin amyloidogenesis, associated with the disease familial amyloidosis of Finnish type, and Pmel17 fiber formation, which is necessary for the functional biogenesis of melanosomes. Proteolysis combined with organelle-dependent environment changes orchestrate amyloidogenesis associated with both pathological processes and a functional pathway.