title: Pathological and functional amyloid formation orchestrated by the secretory pathway.
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.
authors.affiliation: The Scripps Research Institute, Departments of Chemistry, The Skaggs Institute for Chemical Biology, 10550 North Torrey Pines Road, BCC265, La Jolla, CA 92037, USA.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Huff
authors.forename: William E
authors.initials: WE
authors.lastname: Balch
authors.forename: Jeffery W
authors.initials: JW
authors.lastname: Kelly
mesh_terms: D000682:Amyloid / Q000737:chemistry* / Q000378:metabolism*; D000686:Amyloidosis / Q000378:metabolism*; D028226:Amyloidosis, Familial / Q000378:metabolism; D000818:Animals; D018260:Gelsolin / Q000737:chemistry* / Q000378:metabolism*; D006801:Humans; D020460:Melanosomes / Q000378:metabolism; D008562:Membrane Glycoproteins; D011506:Proteins / Q000737:chemistry* / Q000378:metabolism*; D015398:Signal Transduction; D013329:Structure-Activity Relationship; D058951:gp100 Melanoma Antigen
chemical_list: D000682:Amyloid; D018260:Gelsolin; D008562:Membrane Glycoproteins; C548637:PMEL protein, human; D011506:Proteins; D058951:gp100 Melanoma Antigen